European Heart Journal Advance Access published June 26, 2014
REVIEW
European Heart Journal doi:10.1093/eurheartj/ehu248
Clinical update
Ventricular septal rupture complicating acute myocardial infarction: a contemporary review Brandon M. Jones 1, Samir R. Kapadia 1, Nicholas G. Smedira2, Michael Robich 2, E. Murat Tuzcu 1, Venu Menon 1, and Amar Krishnaswamy 1* 1
Received 16 March 2014; revised 17 May 2014; accepted 26 May 2014
Ventricular septal rupture (VSR) after acute myocardial infarction is increasingly rare in the percutaneous coronary intervention era but mortality remains high. Prompt diagnosis is key and definitive surgery, though challenging and associated with high mortality, remains the treatment of choice. Alternatively, delaying surgery in stable patients may provide better results. Prolonged medical management is usually futile, but includes afterload reduction and intra-aortic balloon pump placement. Using full mechanical support to delay surgery is an attractive option, but data on success is limited to case reports. Finally, percutaneous VSR closure may be used as a temporizing measure to reduce shunt, or for patients in the sub-acute to chronic period whose comorbidities preclude surgical repair.
----------------------------------------------------------------------------------------------------------------------------------------------------------Keywords
VSR † Ventricular septal rupture † Acute myocardial infarction † Percutaneous closure
Introduction Ventricular septal rupture (VSR) is a rare but lethal complication of acute myocardial infarction (AMI). As acute reperfusion strategies for AMI have evolved, VSR has become increasingly rare and is identified earlier in the post-MI course. Despite significant improvements over the last two decades in overall mortality for patients with AMI, the outcome of patients who develop VSR remains poor. In this paper, we will review the incidence and pathophysiology of VSR in the current era of aggressive reperfusion with dual antiplatelet therapy and primary percutaneous coronary intervention (PCI), and provide a current perspective on the surgical and percutaneous management of VSR.
Epidemiology The advent of emergent reperfusion strategies for AMI, including thrombolysis and PCI, has led to a decline in the incidence of VSR in contemporary series. Restoration of flow in the infarct related artery leading to myocardial salvage and reduced incidence of transmural infarct may account for this observation. In the prethrombolytic era, VSR was thought to complicate 1–2% of AMI presentations.1 More contemporary series, however, show it to be
increasingly rare, complicating between 0.17 and 0.31% of patients presenting with AMI (Table 1).2 – 5 It is logical to think that mechanical complications would be less frequent in patients treated with primary PCI when compared with thrombolysis or medical therapy alone. In contrast to thrombolysis, optimal PCI results in increased TIMI 3 flow, increased salvage index, and less haemorrhagic transformation of the infarct zone.6 In the Global Registry of Acute Coronary Events (GRACE) registry, patients presenting with ST-elevation MI (STEMI) were evaluated for heart rupture (VSR or free-wall rupture) based on reperfusion strategy. Although neither thrombolytic use nor PCI were independent predictors of heart rupture, there was an overall trend favouring patients treated with PCI (0.7% incidence of heart rupture) vs. those treated with thrombolytics (1.1%) vs. neither lytic or PCI (1.2%).4 There was also a significant linear relationship between shorter time to thrombolysis infusion and lower incidence of heart rupture (P ¼ 0.02).4 Finally, it has been shown that the incidence of VSR is lower in patients who undergo primary PCI compared with those who undergo delayed or elective PCI after recent AMI.7 Thus, strategies aimed at reducing door-to-balloon time for STEMI, including pre-hospital electrocardiogram and bypass of the emergency department, may contribute to the observed reduction in heart rupture.8 Unfortunately though, despite a declining incidence, current mortality among patients with VSR
* Corresponding author. Tel: +1 216 636 2824, Fax: +1 216 445 6153, Email: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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Department of Cardiovascular Medicine, Cleveland Clinic Foundation, 9500 Euclid Ave, Desk J2-3, Cleveland, OH 44195, USA; and Cardiothoracic Surgery, Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA
2
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Table 1 Ventricular septal rupture incidence, time to identification, and resultant mortality in four contemporary clinical series compared with historical data Source
MI treatment (overall cohort)
Incidence of VSR, % (n)
Time to VSR identification
Mortality, %
Historical data
Pre-thrombolysis
1 –2
3 –5 days
With surgery: 45 Without: 90
MIDAS2 1990–2007 n ¼ 148 881 patients with STEMI
PCI (19%)
0.25–0.31 (408)
Not reported
In-hospital: 1990–92: 41 2005–07: 44 1-year: 1990–92: 60 2005–07: 56
GUSTO-13 1990–1993 n ¼ 41 021 patients with STEMI GRACE4 2000–2007 n ¼ 60 198 patients with ‘ACS’
Thrombolysis (100%)
0.20 (84)
1 day (range 0– 47) (94% ≤ 1 week)
30-day: with VSR: 73.8 without VSR: 6.8
Primary PCI (15% overall; 38% for STEMI) Lysis (12% overall; 28% for STEMI)
0.25 (155)
Not reported
In-hospital: 41
APEX-AMI5 2004–2006 n ¼ 5745 patients with STEMI
Primary PCI (94%)
0.17 (10)
7.7 h (range 5.5–23.5)
30-day: 80
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remains high (41–80% in current series), and appears mostly unchanged over the last few decades (Table 1).2,3,5 There has been a steady trend toward earlier identification of VSR after AMI in contemporary trials. While traditionally thought to occur between days 3– 5 after AMI, initial observations from the SHOCK Registry challenged this paradigm, and the median duration of VSR presentation among 55 patients was estimated at 16 h.9 These observations were confirmed in other large clinical series (Table 1).3,5 While these observations may be explained by heightened awareness of VSR and almost universal access to echocardiography, it could be postulated that reperfusion injury combined with fibrinolysis has caused a shift in the underlying pathophysiology, and partially accounts for this finding. There are several independent risk factors for VSR in patients presenting with AMI. These include older age, female sex, prior stroke, chronic kidney disease, and CHF.2,4,9 Patients who develop VSR are more likely to present with ST segment elevation, initial positive cardiac biomarkers, cardiogenic shock, cardiac arrest, higher Killip class, and longer times to first balloon inflation or thrombolytic administration.5,7 Interestingly, patients who develop VSR are less likely to have a history of hypertension, diabetes, prior smoking, or prior MI. It is possible that these patients have pre-existing coronary artery disease, leading to the development of protective collateral circulation, but it is equally possible that these patients develop cardiogenic shock or fatal rupture, leading to observational bias.
Pathophysiology Rupture develops after full-thickness (transmural) infarction of the ventricular septum and can occur at any anatomic location.
Infarctions involving the left-anterior-descending, dominant rightcoronary, or dominant left-circumflex arteries can all involve septal branches. Ventricular septal rupture seems to occur with similar frequency in anterior and inferior/lateral infarctions.2 Anterior infarctions are more likely to cause apical defects and inferior or lateral infarctions are more likely to cause basal defects at the junction of the septum and the posterior wall. Regardless of location, the newly formed communication results in left to right shunting of oxygenated blood from the high-pressure left ventricle (LV) to the lower-pressure right ventricle (RV). Clinical presentation varies from complete haemodynamic stability to frank circulatory collapse depending on the size of the defect, presence of RV infarction, ongoing RV ischaemia, or stunning of the RV from volume overload. Unpredictable haemodynamic deterioration is the norm in most patients in the days and weeks following VSR, and reports of long-term survival independent of corrective surgery are extremely rare. The conventional mechanism of septal rupture involves coagulation necrosis of ischaemic tissue with neutrophilic infiltration, eventually causing thinning and weakening of the septal myocardium.1 This sub-acute process requires 3–5 days, likely accounting for the traditional timing reported in the early surgical literature. Rupture occurring within 24-h of presentation is more likely due to dissection of an intramural haematoma or haemorrhage into ischaemic myocardium. This classically occurs due to physical shear stressors at the border of an infarct zone, combined with a hypercontractile, remote myocardial segment. Clinically, it is most commonly seen in the setting of an inferior infarction with the VSR noted in the basal inferior septum, abutting the hyperdynamic mid-septum that is perfused by the LAD. Ventricular septal rupture can also present in
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VSR, ventricular septal rupture; MI, myocardial infarction; PCI, percutaneous coronary intervention.
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concert with other mechanical complications such as ventricular aneurysm, free wall rupture, or papillary muscle rupture. Becker and Mantgem10 classified the pathological features of cardiac free wall rupture (similar in pathophysiology and therefore applicable to VSR) into three types. Type I rupture shows an abrupt, slit-like tear, and is associated with acute infarcts ,24 h in age. Type II rupture demonstrates erosion of the infarcted myocardium, and correlates clinically with a sub-acute presentation. Type III rupture exhibits concomitant aneurysm formation with significant thinning of the septum and subsequent rupture, a process associated with older infarcts. Septal ruptures are further classified as simple or complex.1 Simple ruptures have a direct connection between the left and right ventricles, occurring at the same level in both chambers, while complex ruptures take a serpigenous course and are more likely to be caused by haemorrhage and irregular tears within the necrotic septum (Figure 1).
Diagnosis It is critical that all patients with AMI have a brief evaluation for mechanical complications prior to primary PCI. All patients who develop haemodynamic compromise during AMI should be rapidly examined for the characteristic, harsh, systolic murmur over the precordium, as well as a palpable thrill, both of which may be difficult to detect in patients with a low output state. Other physical exam findings result from augmented right-sided flow, and may include a loud pulmonic component of the second heart sound, left or right S3 gallop, or tricuspid regurgitation. Left ventriculography performed in the left-anterior oblique (LAO) projection in a patient with VSR may demonstrate shunting of contrast from the LV to the RV. If a PA catheter is placed, the diagnosis should be suspected in patients who have acutely rising mixed Downloaded from http://eurheartj.oxfordjournals.org/ at Carleton University on July 4, 2014
Figure 1 Transthoracic echocardiography demonstrating a simple ventricular septal rupture (arrow) in a patient with mid-left-anterior descending coronary artery infarction in 2D (A) and with colour Doppler (B). Complex ventricular septal rupture in a patient with proximal right coronary artery infarction in 2D (C ) and with colour Doppler (D) showing the ‘serpigenous’ ventricular septal rupture entering the basal left ventricle (arrow) and exiting at multiple points in the apical RV (arrowheads). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
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Surgical management Operative management of patients can be complex, and having a systematic approach is helpful. For an anterior VSR, it is important to assess the severity of LV dysfunction, presence of coexisting CAD, suitability of potential revascularization targets, and anatomy of previous infarctions. For a posterior VSR, an additional understanding of RV function and the presence of mitral regurgitation is important. Most patients with acute post-MI VSR are in cardiogenic shock, and once in the operating room cardiopulmonary bypass with bicaval cannulation should be initiated expeditiously. Many surgeons prefer a left ventriculotomy through the infarct (generally 1 cm lateral to the LAD) to access the VSR as it allows the most direct view of the defect, and infarctectomy or aneurysmectomy can be more easily performed if needed.11 There are two common techniques used for repair of VSR, the Daggett and David procedures (Figure 2). The Daggett is a single or double patch technique which closes the VSR by placing a patch over the defect and sewing to the RV and LV.12 In contrast, the David technique is an infarct exclusion procedure with all sutures placed in the LV.13 In the acute setting, infarcted myocardium is weak and friable, and holds sutures poorly leading to increased risk of tearing and recurrent septal defects. Principles of successful repair include debridement of infarcted tissue back to healthy myocardium (even if it involves enlarging the defect), and avoiding tension on the repair by using an appropriately sized patch.14 Large, 1 cm bites of tissue and buttressing of suture lines with pericardium or felt can improve the strength of the repair. This is especially important when the infarct is ,24 h old as it may be difficult to differentiate viable and infarcted
myocardium visually. In a large VSR there may not be enough anterior septum to allow adequate suturing of the patch. In this case, horizontal mattress sutures can be placed through the right ventricular wall. Posterior VSRs pose additional technical challenges, as the heart must be elevated for adequate exposure. Additionally, the PDA and posteromedial papillary muscle are in close proximity. The transinfarct ventriculotomy is made 1 cm lateral to the PDA and care is taken to preserve the mitral subvalvular apparatus. In both anterior and posterior VSRs, the ventriculotomy is closed primarily or with a patch (usually Dacron) carefully to avoid post-operative free wall rupture. If surgical revascularization is planned, the grafts are placed prior to addressing the VSR to allow for more complete myocardial protection and to minimize handling the heart after the defect is repaired.15 The use of saphenous vein as conduit for all grafts is common.
Outcomes of surgical ventricular septal rupture repair Medical management of VSR is usually futile with rare exception (Figure 3). Definitive surgery remains the treatment of choice, but remains a challenging operation associated with high early mortality. A recently published review of the Society of Thoracic Surgeons National Database (STS Database) identified 2876 individuals aged ≥18 years who underwent post-MI VSR repair between 1999 and 2010.16 Overall operative mortality was 42.9%, which represented the highest mortality rate of any cardiac surgery. Patients who did not survive to 30 days tended to be older, female, had higher serum creatinine levels, and higher acuity of disease (cardiogenic shock, reduced LVEF, triple-vessel CAD, or requirement for preoperative IABP). Operative mortality was much lower for procedures considered elective (13.2% mortality) vs. emergent (56.0% mortality) vs. salvage (80.5% mortality). A smaller study of 68 consecutive patients, 85% of which underwent surgery ,48 h after VSR diagnosis, investigated determinants of post-surgical outcomes.17 In this group, residual interventricular communication was present in 22 of 63 patients (35%) who survived the initial surgery, and was an independent predictor of postoperative CHF. It was due to patch dehiscence in all 11 patients who underwent repeat surgery due to haemodynamic instability (percutaneous closure for residual VSR is discussed subsequently).
Timing of surgery Mortality of patients in the STS database varied significantly depending on timing of surgery. Patients who underwent surgery within 7 days of presentation had a 54.1% mortality compared with 18.4% mortality if repair was delayed until after 7 days. Mortality was highest (.60%) in patients who underwent operation in the first 24 h, consistent with other investigators.18 The improved outcome with delayed surgery may be related to evolution of the infarct and improved stability of the cardiac tissue allowing a more effective repair, but is also a representation of survival bias, as early surgery is usually performed on individuals with marked haemodynamic instability and circulatory compromise. Ultimately, only 886 of the 2876 patients (30.8%) in the STS database
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venous O2 saturations, which could be reflective of a left-to-right shunt. Usually, the diagnosis is made by a prompt transthoracic echocardiogram identifying drop-out of the ventricular septum in the 2D image and demonstration of flow across the septum using colour Doppler (Figure 1). Evidence of right-ventricular dilation and pulmonary hypertension are also important clues to the diagnosis. The remaining portions of the left ventricle are often hyperdynamic unless there is a large territory of infarction, or previous ischaemic insults have led to compromised function. Colour Doppler evaluation can also be useful to assess the anatomical size of the defect. When the patient has poor acoustic windows due to mechanical ventilation or body habitus, a TEE should be considered. When a diagnosis of VSR is made prior to primary PCI, strategies for prompt restoration of coronary flow must be approached with consideration towards the competing need for emergent surgery and the associated risks of surgical bleeding after the administration of dual-antiplatelet therapy. It remains key to limit the ischaemic burden in the infarct-related artery, especially if there is right-ventricular involvement, so immediate collaboration between the interventional cardiologist and cardiac surgeon is critical to develop a case-specific approach. Ideally, VSR would be diagnosed prior to PCI. Flow may be restored in the infarct related artery with aspiration thrombectomy and/or balloon angioplasty, with preparations made for immediate VSR repair with coronary artery bypass grafting. If surgery is delayed, consideration should be given to bare-metal stent placement to minimize dual-antiplatelet therapy duration.
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defect and sewing to the RV and LV (A – C). In contrast, the David technique is an infarct exclusion procedure with all sutures placed in the LV, laying a patch over the VSR to cover the defect (D). The suture line (used to close the ventriculotomy) is buttressed to prevent the stiches from pulling through the infarcted left-ventricular tissue (E). A, aorta; LAD, left-anterior descending coronary artery; PA, pulmonary artery; LV, left ventricle; RV, right ventricle. (Reprinted with permission from Madsen and Daggett.37)
had surgery more than 7 days after presentation, suggesting that a minority of patients survive until elective repair. Also, the number of patients turned down for surgery is unreported. Similarly, the GUSTO-I trial demonstrated a 47% mortality at 30-days among 34 patients who underwent prompt surgical repair (median time 3.5 days, 95% CI, 1–7) vs. 94% mortality for the 35 patients treated without surgery (although again, selection bias must be considered).3 Taken together, the evidence demonstrates that although surgical mortality remains very high, non-surgical mortality is certainly higher. Thus, the clinician must weigh the known risk of expedient surgery against the unknown risk of postponing surgery and developing further clinical deterioration. A simplified version of our own multidisciplinary strategy for managing these patients is included, with the caveat that each case requires a tailored approach (Figure 4).
Medical management and mechanical support The cornerstone of medical management of VSR is afterload reduction to increase effective LV stroke volume by reducing left-to-right shunting. Intravenous afterload-reducing pharmacotherapy such as sodium nitroprusside has the advantage of being rapidly titrated or emergently discontinued compared with oral agents. Intra-aortic balloon counterpulsation (IABP) provides mechanical afterload reduction and augmentation of cardiac output, and may be considered routine care, even in patients who remain haemodynamically stable, as development of haemodynamic compromise is often unexpected, rapid, and fatal. Of the 2876 patients in the STS database, 65% had an IABP placed pre-operatively, and another 8% had an IABP placed
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Figure 2 Daggett vs. David repair of an anterior VSR. The Daggett is a patch technique which closes the VSR by placing a patch directly over the
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Figure 4 A multidisciplinary approach for managing acute ventricular septal rupture.
during surgery. IABP utilization was significantly higher in emergent (84.7%) and salvage (87.9%) cases when compared with elective cases (18.6%).16 Several case studies in the literature have documented the use of extracorporeal membrane oxygenation systems to stabilize patients
until surgery can be performed. Other options include placement of a percutaneous or surgical left-ventricular assist device as a bridge to surgery or transplant.19,20 The data detailing the success of these strategies in patients with acute VSR is currently limited to case reports.21,22
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Figure 3 Reported 30-day mortality for VSR with immediate surgical management or at various time-points of delayed intervention. Mortality is reduced as the time between VSR and surgical intervention increases, likely due to a more stable and fibrosed myocardium, but also due to survival bias. It is unknown what percentage of patients continues to survive as surgery is deferred. In the GUSTO-1 trial, there was 94% mortality at 30 days without surgery suggesting that conservative management is associated with very high mortality. A strategy of full mechanical support allowing for planned, delayed surgery at 7 days may both improve survival while surgery is deferred, and allow for better surgical outcomes due to more stable myocardium, but data are currently limited to case reports.
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Table 2
Major trials of percutaneous closure of ventricular septal rupture
Author
Number of patients
Initial procedural success
Timing of closure (days after AMI)
30-day mortality, %
............................................................................................................................................................................... 30 patients Primary closure: 12 Residual after surgery: 18
Unspecified
19 (11–27) 54 (22–173)
42 11
Maltias,34 2009 Thiele,30 2009
12 patients 29 patients Cardiogenic shock: 16 No shock: 13
Unspecified 25/29
12.2 1 (1–3) 6.5 (4–16)
42 65 88 38
19 patients Primary closure: 17 Residual after surgery: 2 11 patients
14/19
Unspecified
26.3
10/11
2/10 patients: ‘acute’ 8/10 patients: 24–392 25 (2– 95)
100 0 28
Bialkowsk,35 2007
Demkow,36 2005 Holzer,33 2004
18 patients Primary closure: 8 Residual after surgery: 10
16/18
AMI, acute myocardial infarction.
As another strategy, a small series of five patients demonstrated successful use of the Impella 5.0 L system (Abiomed, Danvers, MA, USA) for a mean of 14.4 + 6 days as a bridge to surgery with a 30-day mortality of 40%.23 The TandemHeart pVAD system has been shown to reverse cardiogenic shock in 117 patients, but the cohort included only five patients with acute STEMI, and an unknown number of VSRs.24 Finally, a single case report from Germany documents the use of a total artificial heart followed by a successful cardiac transplant 14 weeks later.19 In the absence of large trial data, these reports provide an interesting, albeit limited, perspective. Short-term mechanical circulatory support as a bridge to recovery is a IIa (LOE C) recommendation in patients with VSR and persistent shock according to current ESC guidelines.25
Percutaneous ventricular septal rupture closure The past decade has witnessed an exciting evolution in structural cardiac interventions.26 – 29 As a result, attention has turned to percutaneous options for the closure of VSR in patients with significant risk for surgical repair, either as a definitive strategy, or as a bridge to surgery after initial stabilization. Several case series have been published documenting single institution experience with both primary percutaneous VSR closure as well as closure of residual defects after surgery (Table 2). Similar to the surgical literature, outcomes improve as patients progress from the acute to chronic phase. The primary goal is a reduction in residual left-to-right shunting (Qp:Qs), which in the largest series of primary VSR closure, was reduced from 3.3 (IQR 2.3– 3.8) to less than 1.5:1 in all but four of 29 patients.30 There are several anatomic concerns in selecting patients for a percutaneous repair.31 Most authors suggest that a defect ,15 mm is optimal, in large part due to the device sizes that are available, as well as the size of the septum. Inferior/posterior defects are especially
challenging as they frequently lack an adequate tissue ‘rim’ to secure the device. The location of the adjoining tricuspid valve apparatus (especially the septal leaflet) makes closure of basal defects more difficult still. Serpigenous defects are not only difficult to cross with a wire, but can be complicated by significant leakage around the device since the path between the ventricles is not straight. Finally, freshly infarcted myocardium may exhibit ongoing necrosis and instability in the days following MI, making peri-device leakage or even device embolization a concern. Thus, attempting device placement requires a thorough understanding of the defect’s size, shape, and borders, usually requiring characterization by both surface and transesophageal echocardiography. Intracardiac echocardiography is invasive and therefore not often used in the screening phase of management; however, it may be useful intra-procedurally, especially when the probe is advanced to the RV. We favour a retrograde approach across the aortic valve (Figure 5). A soft wire (i.e. 0.035′′ Wholey wire) is passed across the VSR from the LV to the RV (along the direction of flow). The wire is then passed to the pulmonary artery to facilitate snaring. The snare may be advanced from the internal jugular or femoral venous approach. While the angle of the SVC to the VSR is more favourable than that of the IVC, we find that the inherent lack of sterility in operating from the neck is a limitation to this approach. Once the wire is snared in the PA, it is externalized. A delivery sheath is then advanced from the venous side and across the VSR into the LV. The intended device is then deployed across the septum. Another novel approach is a combined surgical/percutaneous hybrid closure. Via a standard thoracotomy, a cannulating needle and introducer can be inserted directly into the RV or LV. Under fluoroscopic guidance, the defect is then crossed with a wire and the procedure conducted through the routine steps. Alternatively, a septal occlusion device can be placed under direct visualization. The hybrid approach has the theoretic advantages of easier crossing of the defect and better visualization than a fully percutaneous
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Assenza,32 2013
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strategy, and conversely obviates the need for cardiopulmonary bypass. Furthermore, the surgeon need not suture into freshly infarcted and structurally poor myocardium. Patients who undergo percutaneous closure should receive dual anti-platelet therapy for 6 months followed by baby aspirin alone, and should receive appropriate antibiotic prophylaxis prior to invasive procedures.
requires substantial critical care, imaging, interventional, and surgical expertise. It is therefore advisable, when clinically feasible, to transfer these patients to regional centres with adequate individual experience in the care of patients with VSR.
Conclusions
References
Mortality due to AMI has fallen substantially over the past three decades. Nevertheless, patients who suffer VSR represent a subgroup with extraordinarily high mortality. Management of the patient who is in acute, decompensated cardiogenic shock should be directed at reducing left-to-right shunt with afterload reducing agents and IABP placement. There is no clear evidence to guide the surgical management of patients who are in shock, as all approaches have shown extremely high mortality. Possible strategies include emergent surgery, a period of percutaneous mechanical circulatory support prior to a delayed surgical or percutaneous intervention, or emergent placement of a percutaneous closure device to reduce the shunt. Percutaneous closure may also be a viable option for patients in the sub-acute to chronic period whose comorbidities preclude surgical repair, and whose septal anatomy is favourable to device placement. The management of VSR is complicated, and
Conflict of interest: none declared.
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Figure 5 Percutaneous closure of an apical ventricular septal rupture (VSR). (A) Left ventriculography in the LAO projection demonstrates the apical VSR (arrowhead); (B) a balloon-tipped catheter is passed across the interventricular septum (IVS) (arrowhead) and a gooseneck snare is advanced to the right ventricle (RV, arrow) via the internal jugular (IJ) vein; (C) a wire is passed through the lumen of the balloon catheter, snared, and drawn through to the IJ to provide a rail for device delivery (D); (E) the device is deployed across the IVS; (F) left-ventriculography demonstrates minimal residual shunting of contrast from the LV to the RV.
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37.
myocardial infarct, and a large ventricle septal defect; successful repair after six days. J Cardiac Surg 2013;28:193 –195. La Torre MW, Centofanti P, Attisani M, Patane F, Rinaldi M. Posterior ventricular septal defect in presence of cardiogenic shock: early implantation of the impella recover LP 5.0 as a bridge to surgery. Tex Heart Inst J 2011;38:42– 49. Kar B, Gregoric ID, Basra SS, Idelchik GM, Loyalka P. The percutaneous ventricular assist device in severe refractory cardiogenic shock. J Am Coll Cardiol 2011;57: 688 –696. McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Bohm M, Dickstein K, Falk V, Filippatos G, Fonseca C, Gomez-Sanchez MA, Jaarsma T, Kober L, Lip GY, Maggioni AP, Parkhomenko A, Pieske BM, Popescu BA, Ronnevik PK, Rutten FH, Schwitter J, Seferovic P, Stepinska J, Trindade PT, Voors AA, Zannad F, Zeiher A. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2012: The Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2012 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association (HFA) of the ESC. Eur Heart J 2012;33: 1787 –1847. Krishnaswamy A, Tuzcu EM, Kapadia SR. Update on transcatheter aortic valve implantation. Curr Cardiol Reports 2010;12:393 – 403. Krishnaswamy A, Kapadia SR, Tuzcu EM. Percutaneous paravalvular leak closureimaging, techniques and outcomes. Circ J 2013;77:19–27. Tobis J, Shenoda M. Percutaneous treatment of patent foramen ovale and atrial septal defects. J Am Coll Cardiol 2012;60:1722 –1732. Al-Kashkari W, Balan P, Kavinsky CJ, Cao QL, Hijazi ZM. Percutaneous device closure of congenital and iatrogenic ventricular septal defects in adult patients. Catheter Cardiovasc Interv 2011;77:260 –267. Thiele H, Kaulfersch C, Daehnert I, Schoenauer M, Eitel I, Borger M, Schuler G. Immediate primary transcatheter closure of postinfarction ventricular septal defects. Eur Heart J 2009;30:81 –88. Attia R, Blauth C. Which patients might be suitable for a septal occluder device closure of postinfarction ventricular septal rupture rather than immediate surgery? Interact Cardiovasc Thorac Surg 2010;11:626 – 629. Assenza GE, McElhinney DB, Valente AM, Pearson DD, Volpe M, Martucci G, Landzberg MJ, Lock JE. Transcatheter closure of post-myocardial infarction ventricular septal rupture. Circ Cardiovasc Intervent 2013;6:59 –67. Holzer R, Balzer D, Amin Z, Ruiz CE, Feinstein J, Bass J, Vance M, Cao QL, Hijazi ZM. Transcatheter closure of postinfarction ventricular septal defects using the new Amplatzer muscular VSD occluder: Results of a U.S. Registry. Catheter Cardiovasc Interv 2004;61:196 –201. Maltais S, Ibrahim R, Basmadjian AJ, Carrier M, Bouchard D, Cartier R, Demers P, Ladouceur M, Pellerin M, Perrault LP. Postinfarction ventricular septal defects: towards a new treatment algorithm? Ann Thoracic Surg 2009;87:687 –692. Bialkowski J, Szkutnik M, Zembala M. [Ventricular septal defect closure--importance of cardiac surgery and transcatheter intervention]. Kardiol Pol 2007;65:1022 –1024. Demkow M, Ruzyllo W, Kepka C, Chmielak Z, Konka M, Dzielinska Z, Wilczynski J, Juraszynski Z. Primary transcatheter closure of postinfarction ventricular septal defects with the Amplatzer septal occluder- immediate results and up-to 5 years follow-up. EuroIntervention 2005;1:43–47. Madsen JC, Daggett WM Jr. Repair of postinfarction ventricular septal defects. Semin Thorac Cardiovasc Surg 1998;10:117 –127.
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7. Yip HK, Fang CY, Tsai KT, Chang HW, Yeh KH, Fu M, Wu CJ. The potential impact of primary percutaneous coronary intervention on ventricular septal rupture complicating acute myocardial infarction. Chest 2004;125:1622 –1628. 8. Steg PG, James SK, Atar D, Badano LP, Blomstrom-Lundqvist C, Borger MA, Di Mario C, Dickstein K, Ducrocq G, Fernandez-Aviles F, Gershlick AH, Giannuzzi P, Halvorsen S, Huber K, Juni P, Kastrati A, Knuuti J, Lenzen MJ, Mahaffey KW, Valgimigli M, van ’t Hof A, Widimsky P, Zahger D. ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation. Eur Heart J 2012;33:2569 –2619. 9. Menon V, Webb JG, Hillis LD, Sleeper LA, Abboud R, Dzavik V, Slater JN, Forman R, Monrad ES, Talley JD, Hochman JS. Outcome and profile of ventricular septal rupture with cardiogenic shock after myocardial infarction: a report from the SHOCK Trial Registry. Should we emergently revascularize Occluded Coronaries in cardiogenic shocK? J Am Coll Cardiol 2000;36(Suppl. A):1110– 1116. 10. Becker AE, van Mantgem JP. Cardiac tamponade. A study of 50 hearts. Eur J Cardiol 1975;3:349 –358. 11. Heimbecker RO, Lemire G, Chen C. Surgery for massive myocardial infarction. An experimental study of emergency infarctectomy with a preliminary report on the clinical application. Circulation 1968;37(suppl.):II3–I11. 12. Daggett WM, Guyton RA, Mundth ED, Buckley MJ, McEnany MT, Gold HK, Leinbach RC, Austen WG. Surgery for post-myocardial infarct ventricular septal defect. Ann Surg 1977;186:260 –271. 13. David TE, Dale L, Sun Z. Postinfarction ventricular septal rupture: repair by endocardial patch with infarct exclusion. J Thoracic Cardiovasc Surg 1995;110:1315 –1322. 14. Daggett WM, Buckley MJ, Mundth ED, Sanders CA, Austen WG. The role of infarctectomy in the surgical treatment of myocardial infarction. Am Heart J 1972;84: 723 –726. 15. David TE. Operative management of postinfarction ventricular septal defect. Sem Thoracic Cardiovasc Surg 1995;7:208 –213. 16. Arnaoutakis GJ, Zhao Y, George TJ, Sciortino CM, McCarthy PM, Conte JV. Surgical repair of ventricular septal defect after myocardial infarction: outcomes from the Society of Thoracic Surgeons National Database. Ann Thoracic Surg 2012;94: 436 –443; discussion 443 – 444. 17. Fukushima S, Tesar PJ, Jalali H, Clarke AJ, Sharma H, Choudhary J, Bartlett H, Pohlner PG. Determinants of in-hospital and long-term surgical outcomes after repair of postinfarction ventricular septal rupture. J Thoracic Cardiovasc Surg 2010; 140:59–65. 18. Papalexopoulou N, Young CP, Attia RQ. What is the best timing of surgery in patients with post-infarct ventricular septal rupture? Interactive Cardiovasc Thoracic Surg 2013;16:193 –196. 19. Strueber M, Schmitto JD, Kutschka I, Haverich A. Placement of 2 implantable centrifugal pumps to serve as a total artificial heart after cardiectomy. J Thoracic Cardiovasc Surg 2012;143:507 –509. 20. Loyalka P, Cevik C, Nathan S, Gregoric ID, Kar B. Closure of post-myocardial infarction ventricular septal defect with use of intracardiac echocardiographic imaging and percutaneous left ventricular assistance. Tex Heart Inst J 2012;39:454 –456. 21. Tsai MT, Wu HY, Chan SH, Luo CY. Extracorporeal membrane oxygenation as a bridge to definite surgery in recurrent postinfarction ventricular septal defect. ASAIO J 2012;58:88– 89. 22. Neragi-Miandoab S, Michler RE, Goldstein D, D’Alessandro D. Extracorporeal membrane oxygenation as a temporizing approach in a patient with shock,
REVIEW
European Heart Journal doi:10.1093/eurheartj/ehu248
Clinical update
Ventricular septal rupture complicating acute myocardial infarction: a contemporary review Brandon M. Jones 1, Samir R. Kapadia 1, Nicholas G. Smedira2, Michael Robich 2, E. Murat Tuzcu 1, Venu Menon 1, and Amar Krishnaswamy 1* 1
Received 16 March 2014; revised 17 May 2014; accepted 26 May 2014
Ventricular septal rupture (VSR) after acute myocardial infarction is increasingly rare in the percutaneous coronary intervention era but mortality remains high. Prompt diagnosis is key and definitive surgery, though challenging and associated with high mortality, remains the treatment of choice. Alternatively, delaying surgery in stable patients may provide better results. Prolonged medical management is usually futile, but includes afterload reduction and intra-aortic balloon pump placement. Using full mechanical support to delay surgery is an attractive option, but data on success is limited to case reports. Finally, percutaneous VSR closure may be used as a temporizing measure to reduce shunt, or for patients in the sub-acute to chronic period whose comorbidities preclude surgical repair.
----------------------------------------------------------------------------------------------------------------------------------------------------------Keywords
VSR † Ventricular septal rupture † Acute myocardial infarction † Percutaneous closure
Introduction Ventricular septal rupture (VSR) is a rare but lethal complication of acute myocardial infarction (AMI). As acute reperfusion strategies for AMI have evolved, VSR has become increasingly rare and is identified earlier in the post-MI course. Despite significant improvements over the last two decades in overall mortality for patients with AMI, the outcome of patients who develop VSR remains poor. In this paper, we will review the incidence and pathophysiology of VSR in the current era of aggressive reperfusion with dual antiplatelet therapy and primary percutaneous coronary intervention (PCI), and provide a current perspective on the surgical and percutaneous management of VSR.
Epidemiology The advent of emergent reperfusion strategies for AMI, including thrombolysis and PCI, has led to a decline in the incidence of VSR in contemporary series. Restoration of flow in the infarct related artery leading to myocardial salvage and reduced incidence of transmural infarct may account for this observation. In the prethrombolytic era, VSR was thought to complicate 1–2% of AMI presentations.1 More contemporary series, however, show it to be
increasingly rare, complicating between 0.17 and 0.31% of patients presenting with AMI (Table 1).2 – 5 It is logical to think that mechanical complications would be less frequent in patients treated with primary PCI when compared with thrombolysis or medical therapy alone. In contrast to thrombolysis, optimal PCI results in increased TIMI 3 flow, increased salvage index, and less haemorrhagic transformation of the infarct zone.6 In the Global Registry of Acute Coronary Events (GRACE) registry, patients presenting with ST-elevation MI (STEMI) were evaluated for heart rupture (VSR or free-wall rupture) based on reperfusion strategy. Although neither thrombolytic use nor PCI were independent predictors of heart rupture, there was an overall trend favouring patients treated with PCI (0.7% incidence of heart rupture) vs. those treated with thrombolytics (1.1%) vs. neither lytic or PCI (1.2%).4 There was also a significant linear relationship between shorter time to thrombolysis infusion and lower incidence of heart rupture (P ¼ 0.02).4 Finally, it has been shown that the incidence of VSR is lower in patients who undergo primary PCI compared with those who undergo delayed or elective PCI after recent AMI.7 Thus, strategies aimed at reducing door-to-balloon time for STEMI, including pre-hospital electrocardiogram and bypass of the emergency department, may contribute to the observed reduction in heart rupture.8 Unfortunately though, despite a declining incidence, current mortality among patients with VSR
* Corresponding author. Tel: +1 216 636 2824, Fax: +1 216 445 6153, Email: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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Department of Cardiovascular Medicine, Cleveland Clinic Foundation, 9500 Euclid Ave, Desk J2-3, Cleveland, OH 44195, USA; and Cardiothoracic Surgery, Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA
2
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B.M. Jones et al.
Table 1 Ventricular septal rupture incidence, time to identification, and resultant mortality in four contemporary clinical series compared with historical data Source
MI treatment (overall cohort)
Incidence of VSR, % (n)
Time to VSR identification
Mortality, %
Historical data
Pre-thrombolysis
1 –2
3 –5 days
With surgery: 45 Without: 90
MIDAS2 1990–2007 n ¼ 148 881 patients with STEMI
PCI (19%)
0.25–0.31 (408)
Not reported
In-hospital: 1990–92: 41 2005–07: 44 1-year: 1990–92: 60 2005–07: 56
GUSTO-13 1990–1993 n ¼ 41 021 patients with STEMI GRACE4 2000–2007 n ¼ 60 198 patients with ‘ACS’
Thrombolysis (100%)
0.20 (84)
1 day (range 0– 47) (94% ≤ 1 week)
30-day: with VSR: 73.8 without VSR: 6.8
Primary PCI (15% overall; 38% for STEMI) Lysis (12% overall; 28% for STEMI)
0.25 (155)
Not reported
In-hospital: 41
APEX-AMI5 2004–2006 n ¼ 5745 patients with STEMI
Primary PCI (94%)
0.17 (10)
7.7 h (range 5.5–23.5)
30-day: 80
...............................................................................................................................................................................
remains high (41–80% in current series), and appears mostly unchanged over the last few decades (Table 1).2,3,5 There has been a steady trend toward earlier identification of VSR after AMI in contemporary trials. While traditionally thought to occur between days 3– 5 after AMI, initial observations from the SHOCK Registry challenged this paradigm, and the median duration of VSR presentation among 55 patients was estimated at 16 h.9 These observations were confirmed in other large clinical series (Table 1).3,5 While these observations may be explained by heightened awareness of VSR and almost universal access to echocardiography, it could be postulated that reperfusion injury combined with fibrinolysis has caused a shift in the underlying pathophysiology, and partially accounts for this finding. There are several independent risk factors for VSR in patients presenting with AMI. These include older age, female sex, prior stroke, chronic kidney disease, and CHF.2,4,9 Patients who develop VSR are more likely to present with ST segment elevation, initial positive cardiac biomarkers, cardiogenic shock, cardiac arrest, higher Killip class, and longer times to first balloon inflation or thrombolytic administration.5,7 Interestingly, patients who develop VSR are less likely to have a history of hypertension, diabetes, prior smoking, or prior MI. It is possible that these patients have pre-existing coronary artery disease, leading to the development of protective collateral circulation, but it is equally possible that these patients develop cardiogenic shock or fatal rupture, leading to observational bias.
Pathophysiology Rupture develops after full-thickness (transmural) infarction of the ventricular septum and can occur at any anatomic location.
Infarctions involving the left-anterior-descending, dominant rightcoronary, or dominant left-circumflex arteries can all involve septal branches. Ventricular septal rupture seems to occur with similar frequency in anterior and inferior/lateral infarctions.2 Anterior infarctions are more likely to cause apical defects and inferior or lateral infarctions are more likely to cause basal defects at the junction of the septum and the posterior wall. Regardless of location, the newly formed communication results in left to right shunting of oxygenated blood from the high-pressure left ventricle (LV) to the lower-pressure right ventricle (RV). Clinical presentation varies from complete haemodynamic stability to frank circulatory collapse depending on the size of the defect, presence of RV infarction, ongoing RV ischaemia, or stunning of the RV from volume overload. Unpredictable haemodynamic deterioration is the norm in most patients in the days and weeks following VSR, and reports of long-term survival independent of corrective surgery are extremely rare. The conventional mechanism of septal rupture involves coagulation necrosis of ischaemic tissue with neutrophilic infiltration, eventually causing thinning and weakening of the septal myocardium.1 This sub-acute process requires 3–5 days, likely accounting for the traditional timing reported in the early surgical literature. Rupture occurring within 24-h of presentation is more likely due to dissection of an intramural haematoma or haemorrhage into ischaemic myocardium. This classically occurs due to physical shear stressors at the border of an infarct zone, combined with a hypercontractile, remote myocardial segment. Clinically, it is most commonly seen in the setting of an inferior infarction with the VSR noted in the basal inferior septum, abutting the hyperdynamic mid-septum that is perfused by the LAD. Ventricular septal rupture can also present in
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VSR, ventricular septal rupture; MI, myocardial infarction; PCI, percutaneous coronary intervention.
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concert with other mechanical complications such as ventricular aneurysm, free wall rupture, or papillary muscle rupture. Becker and Mantgem10 classified the pathological features of cardiac free wall rupture (similar in pathophysiology and therefore applicable to VSR) into three types. Type I rupture shows an abrupt, slit-like tear, and is associated with acute infarcts ,24 h in age. Type II rupture demonstrates erosion of the infarcted myocardium, and correlates clinically with a sub-acute presentation. Type III rupture exhibits concomitant aneurysm formation with significant thinning of the septum and subsequent rupture, a process associated with older infarcts. Septal ruptures are further classified as simple or complex.1 Simple ruptures have a direct connection between the left and right ventricles, occurring at the same level in both chambers, while complex ruptures take a serpigenous course and are more likely to be caused by haemorrhage and irregular tears within the necrotic septum (Figure 1).
Diagnosis It is critical that all patients with AMI have a brief evaluation for mechanical complications prior to primary PCI. All patients who develop haemodynamic compromise during AMI should be rapidly examined for the characteristic, harsh, systolic murmur over the precordium, as well as a palpable thrill, both of which may be difficult to detect in patients with a low output state. Other physical exam findings result from augmented right-sided flow, and may include a loud pulmonic component of the second heart sound, left or right S3 gallop, or tricuspid regurgitation. Left ventriculography performed in the left-anterior oblique (LAO) projection in a patient with VSR may demonstrate shunting of contrast from the LV to the RV. If a PA catheter is placed, the diagnosis should be suspected in patients who have acutely rising mixed Downloaded from http://eurheartj.oxfordjournals.org/ at Carleton University on July 4, 2014
Figure 1 Transthoracic echocardiography demonstrating a simple ventricular septal rupture (arrow) in a patient with mid-left-anterior descending coronary artery infarction in 2D (A) and with colour Doppler (B). Complex ventricular septal rupture in a patient with proximal right coronary artery infarction in 2D (C ) and with colour Doppler (D) showing the ‘serpigenous’ ventricular septal rupture entering the basal left ventricle (arrow) and exiting at multiple points in the apical RV (arrowheads). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
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Surgical management Operative management of patients can be complex, and having a systematic approach is helpful. For an anterior VSR, it is important to assess the severity of LV dysfunction, presence of coexisting CAD, suitability of potential revascularization targets, and anatomy of previous infarctions. For a posterior VSR, an additional understanding of RV function and the presence of mitral regurgitation is important. Most patients with acute post-MI VSR are in cardiogenic shock, and once in the operating room cardiopulmonary bypass with bicaval cannulation should be initiated expeditiously. Many surgeons prefer a left ventriculotomy through the infarct (generally 1 cm lateral to the LAD) to access the VSR as it allows the most direct view of the defect, and infarctectomy or aneurysmectomy can be more easily performed if needed.11 There are two common techniques used for repair of VSR, the Daggett and David procedures (Figure 2). The Daggett is a single or double patch technique which closes the VSR by placing a patch over the defect and sewing to the RV and LV.12 In contrast, the David technique is an infarct exclusion procedure with all sutures placed in the LV.13 In the acute setting, infarcted myocardium is weak and friable, and holds sutures poorly leading to increased risk of tearing and recurrent septal defects. Principles of successful repair include debridement of infarcted tissue back to healthy myocardium (even if it involves enlarging the defect), and avoiding tension on the repair by using an appropriately sized patch.14 Large, 1 cm bites of tissue and buttressing of suture lines with pericardium or felt can improve the strength of the repair. This is especially important when the infarct is ,24 h old as it may be difficult to differentiate viable and infarcted
myocardium visually. In a large VSR there may not be enough anterior septum to allow adequate suturing of the patch. In this case, horizontal mattress sutures can be placed through the right ventricular wall. Posterior VSRs pose additional technical challenges, as the heart must be elevated for adequate exposure. Additionally, the PDA and posteromedial papillary muscle are in close proximity. The transinfarct ventriculotomy is made 1 cm lateral to the PDA and care is taken to preserve the mitral subvalvular apparatus. In both anterior and posterior VSRs, the ventriculotomy is closed primarily or with a patch (usually Dacron) carefully to avoid post-operative free wall rupture. If surgical revascularization is planned, the grafts are placed prior to addressing the VSR to allow for more complete myocardial protection and to minimize handling the heart after the defect is repaired.15 The use of saphenous vein as conduit for all grafts is common.
Outcomes of surgical ventricular septal rupture repair Medical management of VSR is usually futile with rare exception (Figure 3). Definitive surgery remains the treatment of choice, but remains a challenging operation associated with high early mortality. A recently published review of the Society of Thoracic Surgeons National Database (STS Database) identified 2876 individuals aged ≥18 years who underwent post-MI VSR repair between 1999 and 2010.16 Overall operative mortality was 42.9%, which represented the highest mortality rate of any cardiac surgery. Patients who did not survive to 30 days tended to be older, female, had higher serum creatinine levels, and higher acuity of disease (cardiogenic shock, reduced LVEF, triple-vessel CAD, or requirement for preoperative IABP). Operative mortality was much lower for procedures considered elective (13.2% mortality) vs. emergent (56.0% mortality) vs. salvage (80.5% mortality). A smaller study of 68 consecutive patients, 85% of which underwent surgery ,48 h after VSR diagnosis, investigated determinants of post-surgical outcomes.17 In this group, residual interventricular communication was present in 22 of 63 patients (35%) who survived the initial surgery, and was an independent predictor of postoperative CHF. It was due to patch dehiscence in all 11 patients who underwent repeat surgery due to haemodynamic instability (percutaneous closure for residual VSR is discussed subsequently).
Timing of surgery Mortality of patients in the STS database varied significantly depending on timing of surgery. Patients who underwent surgery within 7 days of presentation had a 54.1% mortality compared with 18.4% mortality if repair was delayed until after 7 days. Mortality was highest (.60%) in patients who underwent operation in the first 24 h, consistent with other investigators.18 The improved outcome with delayed surgery may be related to evolution of the infarct and improved stability of the cardiac tissue allowing a more effective repair, but is also a representation of survival bias, as early surgery is usually performed on individuals with marked haemodynamic instability and circulatory compromise. Ultimately, only 886 of the 2876 patients (30.8%) in the STS database
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venous O2 saturations, which could be reflective of a left-to-right shunt. Usually, the diagnosis is made by a prompt transthoracic echocardiogram identifying drop-out of the ventricular septum in the 2D image and demonstration of flow across the septum using colour Doppler (Figure 1). Evidence of right-ventricular dilation and pulmonary hypertension are also important clues to the diagnosis. The remaining portions of the left ventricle are often hyperdynamic unless there is a large territory of infarction, or previous ischaemic insults have led to compromised function. Colour Doppler evaluation can also be useful to assess the anatomical size of the defect. When the patient has poor acoustic windows due to mechanical ventilation or body habitus, a TEE should be considered. When a diagnosis of VSR is made prior to primary PCI, strategies for prompt restoration of coronary flow must be approached with consideration towards the competing need for emergent surgery and the associated risks of surgical bleeding after the administration of dual-antiplatelet therapy. It remains key to limit the ischaemic burden in the infarct-related artery, especially if there is right-ventricular involvement, so immediate collaboration between the interventional cardiologist and cardiac surgeon is critical to develop a case-specific approach. Ideally, VSR would be diagnosed prior to PCI. Flow may be restored in the infarct related artery with aspiration thrombectomy and/or balloon angioplasty, with preparations made for immediate VSR repair with coronary artery bypass grafting. If surgery is delayed, consideration should be given to bare-metal stent placement to minimize dual-antiplatelet therapy duration.
B.M. Jones et al.
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defect and sewing to the RV and LV (A – C). In contrast, the David technique is an infarct exclusion procedure with all sutures placed in the LV, laying a patch over the VSR to cover the defect (D). The suture line (used to close the ventriculotomy) is buttressed to prevent the stiches from pulling through the infarcted left-ventricular tissue (E). A, aorta; LAD, left-anterior descending coronary artery; PA, pulmonary artery; LV, left ventricle; RV, right ventricle. (Reprinted with permission from Madsen and Daggett.37)
had surgery more than 7 days after presentation, suggesting that a minority of patients survive until elective repair. Also, the number of patients turned down for surgery is unreported. Similarly, the GUSTO-I trial demonstrated a 47% mortality at 30-days among 34 patients who underwent prompt surgical repair (median time 3.5 days, 95% CI, 1–7) vs. 94% mortality for the 35 patients treated without surgery (although again, selection bias must be considered).3 Taken together, the evidence demonstrates that although surgical mortality remains very high, non-surgical mortality is certainly higher. Thus, the clinician must weigh the known risk of expedient surgery against the unknown risk of postponing surgery and developing further clinical deterioration. A simplified version of our own multidisciplinary strategy for managing these patients is included, with the caveat that each case requires a tailored approach (Figure 4).
Medical management and mechanical support The cornerstone of medical management of VSR is afterload reduction to increase effective LV stroke volume by reducing left-to-right shunting. Intravenous afterload-reducing pharmacotherapy such as sodium nitroprusside has the advantage of being rapidly titrated or emergently discontinued compared with oral agents. Intra-aortic balloon counterpulsation (IABP) provides mechanical afterload reduction and augmentation of cardiac output, and may be considered routine care, even in patients who remain haemodynamically stable, as development of haemodynamic compromise is often unexpected, rapid, and fatal. Of the 2876 patients in the STS database, 65% had an IABP placed pre-operatively, and another 8% had an IABP placed
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Figure 2 Daggett vs. David repair of an anterior VSR. The Daggett is a patch technique which closes the VSR by placing a patch directly over the
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Figure 4 A multidisciplinary approach for managing acute ventricular septal rupture.
during surgery. IABP utilization was significantly higher in emergent (84.7%) and salvage (87.9%) cases when compared with elective cases (18.6%).16 Several case studies in the literature have documented the use of extracorporeal membrane oxygenation systems to stabilize patients
until surgery can be performed. Other options include placement of a percutaneous or surgical left-ventricular assist device as a bridge to surgery or transplant.19,20 The data detailing the success of these strategies in patients with acute VSR is currently limited to case reports.21,22
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Figure 3 Reported 30-day mortality for VSR with immediate surgical management or at various time-points of delayed intervention. Mortality is reduced as the time between VSR and surgical intervention increases, likely due to a more stable and fibrosed myocardium, but also due to survival bias. It is unknown what percentage of patients continues to survive as surgery is deferred. In the GUSTO-1 trial, there was 94% mortality at 30 days without surgery suggesting that conservative management is associated with very high mortality. A strategy of full mechanical support allowing for planned, delayed surgery at 7 days may both improve survival while surgery is deferred, and allow for better surgical outcomes due to more stable myocardium, but data are currently limited to case reports.
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Table 2
Major trials of percutaneous closure of ventricular septal rupture
Author
Number of patients
Initial procedural success
Timing of closure (days after AMI)
30-day mortality, %
............................................................................................................................................................................... 30 patients Primary closure: 12 Residual after surgery: 18
Unspecified
19 (11–27) 54 (22–173)
42 11
Maltias,34 2009 Thiele,30 2009
12 patients 29 patients Cardiogenic shock: 16 No shock: 13
Unspecified 25/29
12.2 1 (1–3) 6.5 (4–16)
42 65 88 38
19 patients Primary closure: 17 Residual after surgery: 2 11 patients
14/19
Unspecified
26.3
10/11
2/10 patients: ‘acute’ 8/10 patients: 24–392 25 (2– 95)
100 0 28
Bialkowsk,35 2007
Demkow,36 2005 Holzer,33 2004
18 patients Primary closure: 8 Residual after surgery: 10
16/18
AMI, acute myocardial infarction.
As another strategy, a small series of five patients demonstrated successful use of the Impella 5.0 L system (Abiomed, Danvers, MA, USA) for a mean of 14.4 + 6 days as a bridge to surgery with a 30-day mortality of 40%.23 The TandemHeart pVAD system has been shown to reverse cardiogenic shock in 117 patients, but the cohort included only five patients with acute STEMI, and an unknown number of VSRs.24 Finally, a single case report from Germany documents the use of a total artificial heart followed by a successful cardiac transplant 14 weeks later.19 In the absence of large trial data, these reports provide an interesting, albeit limited, perspective. Short-term mechanical circulatory support as a bridge to recovery is a IIa (LOE C) recommendation in patients with VSR and persistent shock according to current ESC guidelines.25
Percutaneous ventricular septal rupture closure The past decade has witnessed an exciting evolution in structural cardiac interventions.26 – 29 As a result, attention has turned to percutaneous options for the closure of VSR in patients with significant risk for surgical repair, either as a definitive strategy, or as a bridge to surgery after initial stabilization. Several case series have been published documenting single institution experience with both primary percutaneous VSR closure as well as closure of residual defects after surgery (Table 2). Similar to the surgical literature, outcomes improve as patients progress from the acute to chronic phase. The primary goal is a reduction in residual left-to-right shunting (Qp:Qs), which in the largest series of primary VSR closure, was reduced from 3.3 (IQR 2.3– 3.8) to less than 1.5:1 in all but four of 29 patients.30 There are several anatomic concerns in selecting patients for a percutaneous repair.31 Most authors suggest that a defect ,15 mm is optimal, in large part due to the device sizes that are available, as well as the size of the septum. Inferior/posterior defects are especially
challenging as they frequently lack an adequate tissue ‘rim’ to secure the device. The location of the adjoining tricuspid valve apparatus (especially the septal leaflet) makes closure of basal defects more difficult still. Serpigenous defects are not only difficult to cross with a wire, but can be complicated by significant leakage around the device since the path between the ventricles is not straight. Finally, freshly infarcted myocardium may exhibit ongoing necrosis and instability in the days following MI, making peri-device leakage or even device embolization a concern. Thus, attempting device placement requires a thorough understanding of the defect’s size, shape, and borders, usually requiring characterization by both surface and transesophageal echocardiography. Intracardiac echocardiography is invasive and therefore not often used in the screening phase of management; however, it may be useful intra-procedurally, especially when the probe is advanced to the RV. We favour a retrograde approach across the aortic valve (Figure 5). A soft wire (i.e. 0.035′′ Wholey wire) is passed across the VSR from the LV to the RV (along the direction of flow). The wire is then passed to the pulmonary artery to facilitate snaring. The snare may be advanced from the internal jugular or femoral venous approach. While the angle of the SVC to the VSR is more favourable than that of the IVC, we find that the inherent lack of sterility in operating from the neck is a limitation to this approach. Once the wire is snared in the PA, it is externalized. A delivery sheath is then advanced from the venous side and across the VSR into the LV. The intended device is then deployed across the septum. Another novel approach is a combined surgical/percutaneous hybrid closure. Via a standard thoracotomy, a cannulating needle and introducer can be inserted directly into the RV or LV. Under fluoroscopic guidance, the defect is then crossed with a wire and the procedure conducted through the routine steps. Alternatively, a septal occlusion device can be placed under direct visualization. The hybrid approach has the theoretic advantages of easier crossing of the defect and better visualization than a fully percutaneous
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strategy, and conversely obviates the need for cardiopulmonary bypass. Furthermore, the surgeon need not suture into freshly infarcted and structurally poor myocardium. Patients who undergo percutaneous closure should receive dual anti-platelet therapy for 6 months followed by baby aspirin alone, and should receive appropriate antibiotic prophylaxis prior to invasive procedures.
requires substantial critical care, imaging, interventional, and surgical expertise. It is therefore advisable, when clinically feasible, to transfer these patients to regional centres with adequate individual experience in the care of patients with VSR.
Conclusions
References
Mortality due to AMI has fallen substantially over the past three decades. Nevertheless, patients who suffer VSR represent a subgroup with extraordinarily high mortality. Management of the patient who is in acute, decompensated cardiogenic shock should be directed at reducing left-to-right shunt with afterload reducing agents and IABP placement. There is no clear evidence to guide the surgical management of patients who are in shock, as all approaches have shown extremely high mortality. Possible strategies include emergent surgery, a period of percutaneous mechanical circulatory support prior to a delayed surgical or percutaneous intervention, or emergent placement of a percutaneous closure device to reduce the shunt. Percutaneous closure may also be a viable option for patients in the sub-acute to chronic period whose comorbidities preclude surgical repair, and whose septal anatomy is favourable to device placement. The management of VSR is complicated, and
Conflict of interest: none declared.
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Figure 5 Percutaneous closure of an apical ventricular septal rupture (VSR). (A) Left ventriculography in the LAO projection demonstrates the apical VSR (arrowhead); (B) a balloon-tipped catheter is passed across the interventricular septum (IVS) (arrowhead) and a gooseneck snare is advanced to the right ventricle (RV, arrow) via the internal jugular (IJ) vein; (C) a wire is passed through the lumen of the balloon catheter, snared, and drawn through to the IJ to provide a rail for device delivery (D); (E) the device is deployed across the IVS; (F) left-ventriculography demonstrates minimal residual shunting of contrast from the LV to the RV.
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