2346
CASE REPORT TURIY ET AL ANOMALOUS CORONARY ORIGIN IN HLHS
diaphragm, where adhesions can be particularly dense. Throughout the operation, attention and care should be applied to prevent further traction to the gastrosplenic ligament and disturbing any perisplenic hematoma. It would be advisable to avoid dissection of the short gastric vessels in the construction of a fundoplication. Based on our experience and limited reports in the literature we postulate that traction exerted on the gastrosplenic pedicle against the hernia rim of the diaphragmatic defect, generated during the period of vomiting and retching, may have resulted in an avulsion injury to the spleen. Measures to minimize retching and vomiting should be instituted early in the management, such as nasogastric tube decompression and regular antiemetics. In conclusion, clinicians managing patients with a PEH must not only be aware of this rare complication but also be prepared to intervene in an urgent manner, avoiding life-threatening sequelae.
References
FEATURE ARTICLES
1. Mori T, Nagao G, Sugiyama M. Paraesophageal hernia repair. Ann Thorac Cardiovasc Surg 2012;18:297–305. 2. K€ ohler G, Koch OO, Antoniou SA, Emmanuel K, Pointner R. “Acute intrathoracic stomach!” How should we deal with complicated type IV paraesophageal hernias? Hernia 2014 Jul 25; [Epub ahead of print]. 3. Szwerc MF, Landreneau RJ. Splenic rupture as a consequence of giant paraesophageal hernia. Ann Thorac Surg 2000;70: 1727–8. 4. Polomsky M, Hu R, Sepesi B, et al. A population-based analysis of emergent vs. elective hospital admissions for an intrathoracic stomach. Surg Endosc 2010;24:1250–5.
Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome Yuliya Turiy, MD, MPH, William Douglas, MD, and Duraisamy Balaguru, MD Divisions of Pediatric Cardiology and Pediatric Cardiovascular Surgery, University of Texas Health Science Center, Houston, Texas
We report a case of anomalous origin of the left anterior descending coronary artery (LAD) from the main pulmonary artery in a child with hypoplastic left heart syndrome (mitral atresia/aortic atresia). Mechanical circulatory support was necessary because of the inability to wean from cardiopulmonary bypass after the Norwood procedure. The patient died at 4 months of age having continued depressed right ventricular function. The diagnosis was made during a catheterization performed 6 weeks after surgery because of concern for stenosis of Blalock-Taussig shunt. We believe his prolonged postoperative recovery and eventual demise can partially be Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Ann Thorac Surg 2015;100:2346–8
attributed to lack of cardioplegia to the anomalous LAD territory during surgery. (Ann Thorac Surg 2015;100:2346–8) Ó 2015 by The Society of Thoracic Surgeons
C
oronary artery (CA) anomalies have been reported in small proportion of patients with hypoplastic left heart syndrome (HLHS). An autopsy study of 216 specimens found 49 (22%) that had coronary anomalies: 39 cases of coronary fistulas (all in mitral stenosis and aortic atresia), 9 cases of other CA anomalies such as high takeoff and hypoplasia of CAs and single CA, and only one case of anomalous origin of the left coronary artery (LCA) from the right pulmonary artery (RPA). These latter 10 cases were associated with mitral atresia/aortic atresia anatomy [1]. In addition, several case reports describe anomalous origin of the CA from the RPA in HLHS [2–6]. Anomalies reported include LCA from RPA, circumflex coronary artery from RPA, and right CA from RPA. A large retrospective review of 552 patients with HLHS found 6 patients with anomalous LCA arising from RPA [7]. The association of anomalous origin of the CA from a branch pulmonary artery with HLHS is rare and appears to be more common with the mitral atresia/aortic atresia (MA/AA) type of HLHS. We report a case of HLHS (MA/ AA) associated with anomalous origin of left anterior descending coronary artery (LAD) from the main pulmonary artery. Failure to recognize this associated defect may have contributed to postoperative myocardial dysfunction, the need for mechanical circulatory support, and eventual poor outcome. The patient was born at an outside hospital at term with a birth weight of 2.1 kg. Low oxygen saturations and a heart murmur prompted a hyperoxia test and echocardiography. Diagnosis of HLHS (MA/AA) was made. CAs were well visualized and appeared apparently normal. Prostaglandin infusion was started, and he was transferred to our institution for further management. Because of low birth weight, the patient underwent a modified hybrid procedure with bilateral pulmonary artery banding without complications at 10 days of age. Prostaglandin infusion was continued. A Norwood procedure with 4-mm modified Blalock-Taussig (BT) shunt and debanding of pulmonary arteries was performed at 45 days of age. A polytetrafluoroethylene graft on the innominate artery (eventually used for the BT shunt) was used for arterial cannulation. Cardioplegia was given during a brief period of circulatory arrest with snares on the distal innominate artery (distal to the shunt insertion), left common carotid and subclavian arteries, the ductus arteriosus, and a clamp on the descending aorta. In the operating room, poor right ventricular (RV) systolic Accepted for publication Jan 27, 2015. Address correspondence to Dr Balaguru, University of Texas Health Science Center at Houston, 6410 Fannin St, Houston, TX 77030; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.01.077
Ann Thorac Surg 2015;100:2346–8
CASE REPORT TURIY ET AL ANOMALOUS CORONARY ORIGIN IN HLHS
2347
Fig 1. Posteroanterior (PA) and lateral view aortic root angiograms after Norwood operation with a right-modified Blalock-Taussig shunt. The left anterior descending coronary artery (LAD) originates from neoaorta (Neo). Right coronary artery (RCA) and left circumflex coronary artery (LCX) originate normally from the native aorta (Native).
Comment We report a rare association of anomalous origin of LAD from main pulmonary artery with HLHS. To our knowledge, there is only one other case reported from Japan of a single CA from main pulmonary artery [8]. In this case, the single CA bifurcated into right and left CAs. The patient did not undergo surgery; diagnosis was made at autopsy. In our case, we believe that the
myocardium suffered ischemic damage during the Norwood operation, because the presence of this lesion was not recognized at the time of surgery. Had it been recognized preoperatively or intraoperatively, the administration of cardioplegia to the anomalous LAD apart from routine administration to the CAs arising from native aorta would have prevented myocardial dysfunction at the end of surgery. Because of the inability to wean from cardiopulmonary bypass, ECMO could have been avoided. Since the anomalous LAD originated from main pulmonary artery, which became the neoaorta, there was no need for reimplantation. Myocardial ischemic damage at surgery likely contributed to difficult postoperative course and the eventual death of the child. Although gradual improvement in myocardial function occurred, his RV became significantly dilated and remained so throughout hospitalization. This limited his cardiac reserve, resulting in rapid cardiac decompensation during increased metabolic demand because of conditions such as sepsis. Development of postoperative stenosis in the DKS anastomosis and BT shunt were additional insults. This case underscores the importance of delineating coronary artery anatomy in these patients. Because of the presence of a large patent ductus arteriosus, these anomalies can be missed easily on preoperative echocardiograms. A review of preoperative echocardiograms in our patient shows apparent origin of right and left CAs from respective sinuses of the native aorta. Retrospectively, what appeared to be the LCA was merely a left circumflex branch originating directly from the aortic sinus. Therefore, CA anatomy should be investigated preoperatively with echocardiography and intraoperatively using direct visualization. Although this lesion is rare, awareness of the possibility will enable careful inspection, appropriate modification of operative plan, and administration of cardioplegia, thus avoiding additional potential risk factors in these already high-risk patients. Furthermore, there should be a high index of suspicion for CA abnormalities in patients who have difficulty with myocardial function and perfusion in the operating room and in the immediate postoperative period.
FEATURE ARTICLES
function associated with ST segment elevation was noted, and the patient did not tolerate weaning from cardiopulmonary bypass. Therefore, venoarterial extracorporeal membrane oxygenation (ECMO) was established. Echocardiography showed regional wall motion abnormality of anterior wall of RV. Gradual improvement of RV systolic function occurred, and the patient was weaned off ECMO on the third postoperative day. The patient had a prolonged course in the intensive care unit characterized by multiple infections and an inability to wean from ventilatory support. Approximately 6 weeks after surgery, persistent desaturations were noted. Therefore, he underwent cardiac catheterization where stenoses of the distal end of the BT shunt and Damus-Kaye-Stansel (DKS) anastomosis were noted. Left ventricular end diastolic and right atrial pressures were elevated at 18 mm Hg. A premounted 4.5 12 mm Veriflex MR stent (Boston Scientific, Marlborough, MA) was placed in the distal end of the BT shunt with an improvement in systemic oxygenation. DKS anastomosis had a 9 mm Hg gradient before intervention; it was dilated with a balloon using a 10 20 mm Tyshak Mini balloon (NuMed Inc, Hopkinton, NY) with good results. An aortic root angiogram performed at this catheterization showed anomalous origin of LAD from the neoaorta (Fig 1). Right and left circumflex coronary arteries originated normally from the native aorta. The patient’s oxygen saturations improved, and he tolerated extubation. He was eventually transferred to a step-down unit, where he continued to improve. However, at four months of age, he died after an acute episode of decompensation with severe metabolic acidosis, presumably because of sepsis.
2348
CASE REPORT WILLIAMS ET AL RESECTION OF METASTATIC CARCINOID TUMOR
References 1. Nathan M, Williamson AK, Mayer JE, Bacha EA, Juraszek AL. Mortality in hypoplastic left heart syndrome: review of 216 autopsy cases of aortic atresia with attention to coronary artery disease. J Thoracic Cardiovasc Surg 2012;144: 1301–6. 2. Cleuziou J, Haas F, Schreiber C, Mossinger HJ, Lange R. Hypoplastic left heart syndrome with anomalous origin of the right coronary artery. Ann Thorac Surg 2006;81:341–3. 3. Malec E, Mroczek T, Pajak J, Zajac A, Kolcz J, Januszewska K. Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery. Ann Thorac Surg 2001;72:2129–30. 4. Nosal M, Omeje IC, Poruban R. Hypoplastic left heart syndrome with anomalous origin of left coronary artery from the right pulmonary artery: successful surgical treatment in a neonate. Eur J Cardiothorac Surg 2005;28:497–8. 5. Saroli T, Gelehrter S, Gomez-Fifer CA, Van Der Velde ME, Bove EL, Ensing GJ. Anomalies of left coronary artery origin affecting surgical repair of hypoplastic left heart syndrome and Shone complex. Echocardiography 2008;25: 727–31. 6. Villa E, Brancaccio G, Carotti A, Francalanci P, DiDonato R. Circumflex coronary artery from right pulmonary artery in hypoplastic left heart syndrome. Ann Thorac Surg 2005;80:1919–20. 7. Nathan M, Emani S, Marx G, Pigula F. Anomalous left coronary artery arising from the pulmonary artery in hypoplastic left hearts: case series and review of literature. J Thorac Cardiovasc Surg 2011;142:225–7. 8. Ito T, Niino M, Ishikawa J, et al. Hypoplastic left heart syndrome with a single coronary artery originating from the pulmonary artery. Acta Paediatr Jpn 1995;37:61–3.
Resection of Carcinoid Tumor Metastatic to the Right Ventricle and Tricuspid Valve FEATURE ARTICLES
Adam R. Williams, MD, Andrew Wang, MD, Han Kim, MD, and Carmelo A. Milano, MD Departments of Surgery and Medicine, Duke University, Durham, North Carolina
Carcinoid tumors rarely metastasize to the heart, and previous reports involved the myocardium only. We present a case of carcinoid tumor metastatic to the right ventricle (RV) and tricuspid valve. Preoperative cardiac magnetic resonance imaging (CMR) showed the tumor arising from the right ventricular free wall and involving the tricuspid valve apparatus. The tumor was resected through a right atriotomy and required tricuspid valve replacement. (Ann Thorac Surg 2015;100:2348–50) Ó 2015 by The Society of Thoracic Surgeons
C
arcinoid tumors typically originate in the gastrointestinal tract and secrete serotonin, a hormone that causes right-sided valve destruction [1]. These tumors are Accepted for publication Feb 6, 2015. Address correspondence to Dr Milano, Division of Thoracic Surgery, Duke University, DUMC 3043, Durham, NC 27710; e-mail: carmelo. [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Ann Thorac Surg 2015;100:2348–50
capable of metastatic spread to other organs, most commonly the liver; however, they rarely metastasize to the heart [2]. We present a case of an adult woman with a carcinoid tumor metastatic to the right ventricle (RV) and involving the tricuspid valve. We also describe the surgical technique of tumor resection. A 42-year-old woman presented with abdominal pain, episodes of flushing, and diarrhea. A computed tomographic scan demonstrated a well-circumscribed tumor in her liver and a second tumor in the RV. Percutaneous biopsy of the heart demonstrated myxomatous changes; however, the liver biopsy results were consistent with carcinoid tumor. Colonoscopy identified a primary carcinoid tumor in the right colon. Transthoracic echocardiography showed a large mass in the RV measuring 6 6 4.4 cm obstructing blood flow through the right side of the heart. Cardiac magnetic resonance imaging (CMR) demonstrated involvement of the septal and posterior tricuspid leaflets as well as the chordal apparatus (Fig 1A). CMR also showed a capsule around the mass with a distinct tumor border from the interventricular septum (Fig 1B). Left heart catheterization showed branches of the right coronary artery feeding a highly vascular tumor (Fig 1C). The patient was given subcutaneous octreotide injections for several months before the surgical procedure to control the symptoms of carcinoid syndrome. Consultation with the gastrointestinal surgery department indicated that the hepatic and colonic lesions were resectable. Because of symptoms of right-sided heart failure and the appearance of right ventricular obstruction on CMR, the decision was made to offer the patient an exploratory operation and resection. The decision for a cardiac operation was also supported by the CMR study that showed a well-circumscribed lesion involving the tricuspid valve but not invading the myocardium. Intravenous infusion of octreotide was initiated at 25 mg/h beginning the day of the operation and continued through postoperative day 2 to prevent a carcinoid storm. She was taken to the operating room for resection of the metastatic cardiac tumor with plans for later staged resection of the liver and gastrointestinal lesions. A median sternotomy was performed with standard aortic arterial and bicaval venous cannulation. Inferior vena cava (IVC) cannulation was difficult because of distortion of the IVC by the tumor mass. Cardiopulmonary bypass was initiated and the heart was arrested with antegrade cardioplegia. The right atrium was opened, and a small patent foramen ovale was closed with interrupted sutures. The tumor was inspected and deemed to be resectable through the tricuspid valve (Fig 2). Because the tricuspid valve was extensively stretched and tethered by the tumor, the decision was made to resect the valve. The tumor was excised from the right ventricular free wall and septum using electrocautery. A fibrous shell appeared to separate the tumor from distorted myocardium. The tumor was shelled out in its entirety, and the tricuspid valve was then replaced using a 31mm Medtronic Mosaic Tissue Valve (Medtronic, 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.108
CASE REPORT TURIY ET AL ANOMALOUS CORONARY ORIGIN IN HLHS
diaphragm, where adhesions can be particularly dense. Throughout the operation, attention and care should be applied to prevent further traction to the gastrosplenic ligament and disturbing any perisplenic hematoma. It would be advisable to avoid dissection of the short gastric vessels in the construction of a fundoplication. Based on our experience and limited reports in the literature we postulate that traction exerted on the gastrosplenic pedicle against the hernia rim of the diaphragmatic defect, generated during the period of vomiting and retching, may have resulted in an avulsion injury to the spleen. Measures to minimize retching and vomiting should be instituted early in the management, such as nasogastric tube decompression and regular antiemetics. In conclusion, clinicians managing patients with a PEH must not only be aware of this rare complication but also be prepared to intervene in an urgent manner, avoiding life-threatening sequelae.
References
FEATURE ARTICLES
1. Mori T, Nagao G, Sugiyama M. Paraesophageal hernia repair. Ann Thorac Cardiovasc Surg 2012;18:297–305. 2. K€ ohler G, Koch OO, Antoniou SA, Emmanuel K, Pointner R. “Acute intrathoracic stomach!” How should we deal with complicated type IV paraesophageal hernias? Hernia 2014 Jul 25; [Epub ahead of print]. 3. Szwerc MF, Landreneau RJ. Splenic rupture as a consequence of giant paraesophageal hernia. Ann Thorac Surg 2000;70: 1727–8. 4. Polomsky M, Hu R, Sepesi B, et al. A population-based analysis of emergent vs. elective hospital admissions for an intrathoracic stomach. Surg Endosc 2010;24:1250–5.
Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome Yuliya Turiy, MD, MPH, William Douglas, MD, and Duraisamy Balaguru, MD Divisions of Pediatric Cardiology and Pediatric Cardiovascular Surgery, University of Texas Health Science Center, Houston, Texas
We report a case of anomalous origin of the left anterior descending coronary artery (LAD) from the main pulmonary artery in a child with hypoplastic left heart syndrome (mitral atresia/aortic atresia). Mechanical circulatory support was necessary because of the inability to wean from cardiopulmonary bypass after the Norwood procedure. The patient died at 4 months of age having continued depressed right ventricular function. The diagnosis was made during a catheterization performed 6 weeks after surgery because of concern for stenosis of Blalock-Taussig shunt. We believe his prolonged postoperative recovery and eventual demise can partially be Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Ann Thorac Surg 2015;100:2346–8
attributed to lack of cardioplegia to the anomalous LAD territory during surgery. (Ann Thorac Surg 2015;100:2346–8) Ó 2015 by The Society of Thoracic Surgeons
C
oronary artery (CA) anomalies have been reported in small proportion of patients with hypoplastic left heart syndrome (HLHS). An autopsy study of 216 specimens found 49 (22%) that had coronary anomalies: 39 cases of coronary fistulas (all in mitral stenosis and aortic atresia), 9 cases of other CA anomalies such as high takeoff and hypoplasia of CAs and single CA, and only one case of anomalous origin of the left coronary artery (LCA) from the right pulmonary artery (RPA). These latter 10 cases were associated with mitral atresia/aortic atresia anatomy [1]. In addition, several case reports describe anomalous origin of the CA from the RPA in HLHS [2–6]. Anomalies reported include LCA from RPA, circumflex coronary artery from RPA, and right CA from RPA. A large retrospective review of 552 patients with HLHS found 6 patients with anomalous LCA arising from RPA [7]. The association of anomalous origin of the CA from a branch pulmonary artery with HLHS is rare and appears to be more common with the mitral atresia/aortic atresia (MA/AA) type of HLHS. We report a case of HLHS (MA/ AA) associated with anomalous origin of left anterior descending coronary artery (LAD) from the main pulmonary artery. Failure to recognize this associated defect may have contributed to postoperative myocardial dysfunction, the need for mechanical circulatory support, and eventual poor outcome. The patient was born at an outside hospital at term with a birth weight of 2.1 kg. Low oxygen saturations and a heart murmur prompted a hyperoxia test and echocardiography. Diagnosis of HLHS (MA/AA) was made. CAs were well visualized and appeared apparently normal. Prostaglandin infusion was started, and he was transferred to our institution for further management. Because of low birth weight, the patient underwent a modified hybrid procedure with bilateral pulmonary artery banding without complications at 10 days of age. Prostaglandin infusion was continued. A Norwood procedure with 4-mm modified Blalock-Taussig (BT) shunt and debanding of pulmonary arteries was performed at 45 days of age. A polytetrafluoroethylene graft on the innominate artery (eventually used for the BT shunt) was used for arterial cannulation. Cardioplegia was given during a brief period of circulatory arrest with snares on the distal innominate artery (distal to the shunt insertion), left common carotid and subclavian arteries, the ductus arteriosus, and a clamp on the descending aorta. In the operating room, poor right ventricular (RV) systolic Accepted for publication Jan 27, 2015. Address correspondence to Dr Balaguru, University of Texas Health Science Center at Houston, 6410 Fannin St, Houston, TX 77030; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.01.077
Ann Thorac Surg 2015;100:2346–8
CASE REPORT TURIY ET AL ANOMALOUS CORONARY ORIGIN IN HLHS
2347
Fig 1. Posteroanterior (PA) and lateral view aortic root angiograms after Norwood operation with a right-modified Blalock-Taussig shunt. The left anterior descending coronary artery (LAD) originates from neoaorta (Neo). Right coronary artery (RCA) and left circumflex coronary artery (LCX) originate normally from the native aorta (Native).
Comment We report a rare association of anomalous origin of LAD from main pulmonary artery with HLHS. To our knowledge, there is only one other case reported from Japan of a single CA from main pulmonary artery [8]. In this case, the single CA bifurcated into right and left CAs. The patient did not undergo surgery; diagnosis was made at autopsy. In our case, we believe that the
myocardium suffered ischemic damage during the Norwood operation, because the presence of this lesion was not recognized at the time of surgery. Had it been recognized preoperatively or intraoperatively, the administration of cardioplegia to the anomalous LAD apart from routine administration to the CAs arising from native aorta would have prevented myocardial dysfunction at the end of surgery. Because of the inability to wean from cardiopulmonary bypass, ECMO could have been avoided. Since the anomalous LAD originated from main pulmonary artery, which became the neoaorta, there was no need for reimplantation. Myocardial ischemic damage at surgery likely contributed to difficult postoperative course and the eventual death of the child. Although gradual improvement in myocardial function occurred, his RV became significantly dilated and remained so throughout hospitalization. This limited his cardiac reserve, resulting in rapid cardiac decompensation during increased metabolic demand because of conditions such as sepsis. Development of postoperative stenosis in the DKS anastomosis and BT shunt were additional insults. This case underscores the importance of delineating coronary artery anatomy in these patients. Because of the presence of a large patent ductus arteriosus, these anomalies can be missed easily on preoperative echocardiograms. A review of preoperative echocardiograms in our patient shows apparent origin of right and left CAs from respective sinuses of the native aorta. Retrospectively, what appeared to be the LCA was merely a left circumflex branch originating directly from the aortic sinus. Therefore, CA anatomy should be investigated preoperatively with echocardiography and intraoperatively using direct visualization. Although this lesion is rare, awareness of the possibility will enable careful inspection, appropriate modification of operative plan, and administration of cardioplegia, thus avoiding additional potential risk factors in these already high-risk patients. Furthermore, there should be a high index of suspicion for CA abnormalities in patients who have difficulty with myocardial function and perfusion in the operating room and in the immediate postoperative period.
FEATURE ARTICLES
function associated with ST segment elevation was noted, and the patient did not tolerate weaning from cardiopulmonary bypass. Therefore, venoarterial extracorporeal membrane oxygenation (ECMO) was established. Echocardiography showed regional wall motion abnormality of anterior wall of RV. Gradual improvement of RV systolic function occurred, and the patient was weaned off ECMO on the third postoperative day. The patient had a prolonged course in the intensive care unit characterized by multiple infections and an inability to wean from ventilatory support. Approximately 6 weeks after surgery, persistent desaturations were noted. Therefore, he underwent cardiac catheterization where stenoses of the distal end of the BT shunt and Damus-Kaye-Stansel (DKS) anastomosis were noted. Left ventricular end diastolic and right atrial pressures were elevated at 18 mm Hg. A premounted 4.5 12 mm Veriflex MR stent (Boston Scientific, Marlborough, MA) was placed in the distal end of the BT shunt with an improvement in systemic oxygenation. DKS anastomosis had a 9 mm Hg gradient before intervention; it was dilated with a balloon using a 10 20 mm Tyshak Mini balloon (NuMed Inc, Hopkinton, NY) with good results. An aortic root angiogram performed at this catheterization showed anomalous origin of LAD from the neoaorta (Fig 1). Right and left circumflex coronary arteries originated normally from the native aorta. The patient’s oxygen saturations improved, and he tolerated extubation. He was eventually transferred to a step-down unit, where he continued to improve. However, at four months of age, he died after an acute episode of decompensation with severe metabolic acidosis, presumably because of sepsis.
2348
CASE REPORT WILLIAMS ET AL RESECTION OF METASTATIC CARCINOID TUMOR
References 1. Nathan M, Williamson AK, Mayer JE, Bacha EA, Juraszek AL. Mortality in hypoplastic left heart syndrome: review of 216 autopsy cases of aortic atresia with attention to coronary artery disease. J Thoracic Cardiovasc Surg 2012;144: 1301–6. 2. Cleuziou J, Haas F, Schreiber C, Mossinger HJ, Lange R. Hypoplastic left heart syndrome with anomalous origin of the right coronary artery. Ann Thorac Surg 2006;81:341–3. 3. Malec E, Mroczek T, Pajak J, Zajac A, Kolcz J, Januszewska K. Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery. Ann Thorac Surg 2001;72:2129–30. 4. Nosal M, Omeje IC, Poruban R. Hypoplastic left heart syndrome with anomalous origin of left coronary artery from the right pulmonary artery: successful surgical treatment in a neonate. Eur J Cardiothorac Surg 2005;28:497–8. 5. Saroli T, Gelehrter S, Gomez-Fifer CA, Van Der Velde ME, Bove EL, Ensing GJ. Anomalies of left coronary artery origin affecting surgical repair of hypoplastic left heart syndrome and Shone complex. Echocardiography 2008;25: 727–31. 6. Villa E, Brancaccio G, Carotti A, Francalanci P, DiDonato R. Circumflex coronary artery from right pulmonary artery in hypoplastic left heart syndrome. Ann Thorac Surg 2005;80:1919–20. 7. Nathan M, Emani S, Marx G, Pigula F. Anomalous left coronary artery arising from the pulmonary artery in hypoplastic left hearts: case series and review of literature. J Thorac Cardiovasc Surg 2011;142:225–7. 8. Ito T, Niino M, Ishikawa J, et al. Hypoplastic left heart syndrome with a single coronary artery originating from the pulmonary artery. Acta Paediatr Jpn 1995;37:61–3.
Resection of Carcinoid Tumor Metastatic to the Right Ventricle and Tricuspid Valve FEATURE ARTICLES
Adam R. Williams, MD, Andrew Wang, MD, Han Kim, MD, and Carmelo A. Milano, MD Departments of Surgery and Medicine, Duke University, Durham, North Carolina
Carcinoid tumors rarely metastasize to the heart, and previous reports involved the myocardium only. We present a case of carcinoid tumor metastatic to the right ventricle (RV) and tricuspid valve. Preoperative cardiac magnetic resonance imaging (CMR) showed the tumor arising from the right ventricular free wall and involving the tricuspid valve apparatus. The tumor was resected through a right atriotomy and required tricuspid valve replacement. (Ann Thorac Surg 2015;100:2348–50) Ó 2015 by The Society of Thoracic Surgeons
C
arcinoid tumors typically originate in the gastrointestinal tract and secrete serotonin, a hormone that causes right-sided valve destruction [1]. These tumors are Accepted for publication Feb 6, 2015. Address correspondence to Dr Milano, Division of Thoracic Surgery, Duke University, DUMC 3043, Durham, NC 27710; e-mail: carmelo. [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Ann Thorac Surg 2015;100:2348–50
capable of metastatic spread to other organs, most commonly the liver; however, they rarely metastasize to the heart [2]. We present a case of an adult woman with a carcinoid tumor metastatic to the right ventricle (RV) and involving the tricuspid valve. We also describe the surgical technique of tumor resection. A 42-year-old woman presented with abdominal pain, episodes of flushing, and diarrhea. A computed tomographic scan demonstrated a well-circumscribed tumor in her liver and a second tumor in the RV. Percutaneous biopsy of the heart demonstrated myxomatous changes; however, the liver biopsy results were consistent with carcinoid tumor. Colonoscopy identified a primary carcinoid tumor in the right colon. Transthoracic echocardiography showed a large mass in the RV measuring 6 6 4.4 cm obstructing blood flow through the right side of the heart. Cardiac magnetic resonance imaging (CMR) demonstrated involvement of the septal and posterior tricuspid leaflets as well as the chordal apparatus (Fig 1A). CMR also showed a capsule around the mass with a distinct tumor border from the interventricular septum (Fig 1B). Left heart catheterization showed branches of the right coronary artery feeding a highly vascular tumor (Fig 1C). The patient was given subcutaneous octreotide injections for several months before the surgical procedure to control the symptoms of carcinoid syndrome. Consultation with the gastrointestinal surgery department indicated that the hepatic and colonic lesions were resectable. Because of symptoms of right-sided heart failure and the appearance of right ventricular obstruction on CMR, the decision was made to offer the patient an exploratory operation and resection. The decision for a cardiac operation was also supported by the CMR study that showed a well-circumscribed lesion involving the tricuspid valve but not invading the myocardium. Intravenous infusion of octreotide was initiated at 25 mg/h beginning the day of the operation and continued through postoperative day 2 to prevent a carcinoid storm. She was taken to the operating room for resection of the metastatic cardiac tumor with plans for later staged resection of the liver and gastrointestinal lesions. A median sternotomy was performed with standard aortic arterial and bicaval venous cannulation. Inferior vena cava (IVC) cannulation was difficult because of distortion of the IVC by the tumor mass. Cardiopulmonary bypass was initiated and the heart was arrested with antegrade cardioplegia. The right atrium was opened, and a small patent foramen ovale was closed with interrupted sutures. The tumor was inspected and deemed to be resectable through the tricuspid valve (Fig 2). Because the tricuspid valve was extensively stretched and tethered by the tumor, the decision was made to resect the valve. The tumor was excised from the right ventricular free wall and septum using electrocautery. A fibrous shell appeared to separate the tumor from distorted myocardium. The tumor was shelled out in its entirety, and the tricuspid valve was then replaced using a 31mm Medtronic Mosaic Tissue Valve (Medtronic, 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.108
