© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12781
Echocardiography
Prenatal Diagnosis of Giant Left Ventricular Diverticulum: Case Report ur Gocen, M.D.,† and Selim Buyukkurt, M.D.‡ Fadli Demir, M.D.,* Nazan Ozbarlas, M.D.,* Ug *Department of Pediatric Cardiology, C ß ukurova University School of Medicine, Adana, Turkey; †Department of Cardiovascular Surgery, C ß ukurova University School of Medicine, Adana, Turkey; and ‡Department of Obstetrics and Gynecology, C ß ukurova University School of Medicine, Adana, Turkey
The distinction between a ventricular aneurysm and diverticulum is difficult. The differences between these two are based on anatomical, histological, and functional criteria. We present a case of prenatal diagnosis of a giant left ventricular outpouching at 28 weeks gestation. During the postnatal period, the neonate underwent surgical correction for the outpouching. Histopathological examination reported the resected segment was a cardiac diverticulum. Our case indicates that differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. Histopathological examinations are sometimes necessary to make a precise differentiation. Treatment should be individualized depending on the clinical presentation. (Echocardiography 2015;32:395–397) Key words: prenatal diagnosis, diverticula, fetal echocardiography, left ventricular shape
A congenital ventricular diverticulum (CVD) and aneurysm are both localized protrusions within the free wall or apex of one of the heart ventricles. Diagnosing CVD during the fetal period is very rare. CVDs usually arise from the left ventricle (LV).1,2 Although CVD etiology is not clear, an embryological developmental defect has been proposed.3 CVDs are often clinically very difficult to distinguish from a congenital aneurysm.4,5 Anatomical, histological, and functional criteria are used to differentiate between a diverticulum and an aneurysm.1,6–8 Herein, we describe a rare case of a wide-mouthed LV free wall outpouching that was identified by fetal echocardiography. Case: A 37-year-old woman (G4P3A1L3) was referred for fetal echocardiography after the detection of cardiomegaly on the comprehensive obstetric ultrasound screening. Fetal echocardiography performed at the 28th week of pregnancy showed two ventricles with normal atrioventricular and ventriculoarterial concordance. Apical four- and apical two-chamber views showed a large LV free wall outpouching (Fig. 1). The outpouching measured 30 9 14 mm in diameter; Address for correspondence and reprint requests: Fadli Demir, M.D., Department of Pediatric Cardiology, C ß ukurova University School of Medicine, 01330 Balcali, Sarıcßam, Adana, Turkey. Fax: +90 322 338 69 45; E-mail: [email protected]
the mouth of the outpouching measured 16 mm (Fig. 2). The diastolic volume of the outpouching was 5.8 mL and the ratio of the volume of the outpouching to the diastolic LV volume was 1.4. A very small pericardial effusion was seen. The thickness and acoustic properties of the outpouching wall was similar to those of the LV wall (Fig. 3). No other cardiac or extracardiac anomalies were detected. The mitral valve apparatus appeared normal. No arrhythmias or intracardiac thrombi were seen. The patient was evaluated every 4 weeks by serial echocardiograms. No changes were noted in either the size or the function of the LV and the outpouching. Over the following weeks, pericardial effusion resolved spontaneously. The baby was born at term by normal vaginal delivery and weighed 3010 g. Postnatal echocardiography showed findings similar to the fetal echocardiography: a large LV free wall outpouching with a maximum diameter of 41 9 23 mm and a wide mouth seen in Figure 4. Additionally, spontaneous echo contrast was seen in the outpouching. Measured LV ejection fraction was 55%. Outpouching showed synchrony with the interventricular septum. The patient was subsequently transferred to the neonatal intensive care unit. Upon admission, the baby had mild tachypnea; dopamine, captopril, aspirin, and low-molecular-weight heparin were started. Tachypnea and clinical findings consistent with heart failure did not subside, therefore, the Dor procedure was performed at 17 days of 395
Demir, et al.
age. During this procedure, LV aneurysmal tissue was resected with endoventricular reductoplasty, using a Dacron patch (Figs. 5 and 6). The postoperative recovery period was uneventful. A histopathological examination reported the resected segment as a cardiac diverticulum. The patient is currently 4 months old and in good general health. Conclusion: The advancement of prenatal diagnostic methods has led to prenatal early diagnosis of many rare cardiac abnormalities, such as diverticula and aneurysms. The reasons for referral for fetal echocardiography in most cases have varied from cardiomegaly to hydrops and pericardial effusion to arrhythmias seen during routine prenatal ultrasound examinations.1,2,4,6,7 Our patient was referred for cardiomegaly. The distinction between CVDs and aneurysms is important because the treatment and prognosis can differ. CVDs are synchronized with the rest of the ventricles and involve a narrow connection with the ventricular cavity. CVDs display a similar thickness and layering as the rest of the ventricular myocardium. Apical lesions have been associated with midline thoracoabdominal defects and with other cardiac malformations. Unlike CVDs, cardiac aneurysms have a broad base, appear hypokinetic, and move paradoxically to the cardiac rhythm. Congenital LV aneurysms are not typically associated with other cardiac anomalies.3,4,9 In our case, the presence of a large communication with the LV and the absence of associated cardiac and extracardiac anomalies favor the diagnosis of a ventricular aneurysm. However, synchronous contractions of the outpouching with the LV supported the diagnosis of a diverticulum. Additionally, histopathological diagnosis supported our assumption of a diverticulum.
Figure 2. Fetal echocardiogram at 32 weeks gestation. Arrow displays the left ventricular outpouching. RV = right ventricle; LV = left ventricle.
Figure 3. Intrauterine M-mode. Outpouching (black arrow) is syncronous contracting with IVS (white arrow). RV = right ventricle; LV = left ventricle; IVS = interventricular septum.
Figure 4. Postnatal echocardiography showing a markedly enlarged LV outpouching (arrow). LV = left ventricle; IVS = interventricular septum.
Figure 1. Four-chamber view fetal echocardiogram at 28 weeks gestation, showing a left ventricular outpouching (arrow).
396
The size of the CVD usually remains stable or decreases relative to the ventricle during fetal development. This natural history of the CVD is in contrast with that of a cardiac aneurysm, where enlargement throughout the pregnancy is observed.10 This increase in size is important
Giant Left Ventricular Diverticulum
A
B
Figure 5. A. Intra-operative view of the outpouching (arrow). B. Ventricular restoration using a Dacron patch (arrow).
Figure 6. Postoperative echocardiography showing a restored LV geometry and decreased LV dimension after LV diverticulum resection with endoventricular reductoplasty, using a Dacron patch (arrow). LV = left ventricle.
the outpouching demonstrated spontaneous echo contrast, which is considered an advance finding for thrombus development. In this case, the patient was symptomatic with an outpouching too large to permit proper LV function. Through the use of the Dor procedure, we expected an improvement in LV geometry and LV function. In conclusion, a fetal cardiac diverticulum and aneurysm are rare cardiac anomalies. Differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. The postnatal course of the patient may aid in the differential diagnosis; however, sometimes histopathological examination is necessary to make a precise differentiation. References
because the outcome of a fetal aneurysm is related to the ratio of volume between the aneurysm and the ventricular cavity.2–4,6 The size of the CVD in our patient remained stable throughout the pregnancy. Patients with a CVD that is not associated with other malformations are usually asymptomatic. However, some fetuses may develop an impairment of LV function, pericardial effusions, acute rupture, and fetal arrhythmias.1–3 In our case, these complications were not observed during follow-up examinations. Treatment of CVDs and aneurysms are dependent on the patient’s clinical status, presence of other cardiac abnormalities, and the size of the outpouching. Most CVD patients are asymptomatic at birth and there is a decrease in the size of the diverticulum. Patients with CVDs that are symptomatic and associated with other malformations usually require surgical intervention. The prognosis for congenital ventricular aneurysms is related to the size, course, and location of the aneurysm, degree of mitral valve involvement, associated heart failure, arrhythmias, and the presence of a thrombus.1,3,4,6 Early surgical intervention was performed in our patient since the size of the outpouching was substantial and
1. Bernasconi A, Delezoide AL, Menez F, et al: Prenatal rupture of a left ventricular diverticulum: A case report and review of the literature. Prenat Diagn 2004;24:504–507. 2. Perlitz Y, Mukary M, Lorber A, et al: Prenatal diagnosis of fetal cardiac right ventricular diverticulum disappearing at three months of age a case report and literature review. Fetal Diagn Ther 2009;25:44–46. 3. Giannoglou GD, Delioglanis SH, Paraskevaidis S, et al: Ventricular diverticulum: Definition, pathophysiology, clinical manifestations and treatment. Aristotle Univ Med J 2007;34:87–92. 4. Giacobbe L, Williams P, Ramin K, et al: Fetal diagnosis of left ventricular aneurysm: A case report. Am J Perinatol Rep 2011;1:91–94. 5. Awad SM, Patel AS, Polimenakos A, et al: Left ventricular accessory chamber: A case report and review of the literature. Pediatr Cardiol 2009;30:1022–1025. 6. Barberato MF, Barberato SH, Binotto CN, et al: Prenatal diagnosis of left ventricular aneurysm and diverticulum. Arq Bras Cardiol 2009;93:24–26. 7. Paoletti D, Robertson M: Prenatal diagnosis of a left ventricular diverticulum. AJUM 2012;15:112–114. 8. Ruiz-Esparza E, Roldan FJ, Vanquez-Antona C, et al: 2D and 3D echocardiography of left ventricular diverticulum. Echocardiography 2009;26:1087–1088. 9. McAuliffe FM, Hornberger LK, Johnson J, et al: Cardiac diverticulum with pericardial effusion: Report of two new cases treated by in-utero pericardiocentesis and a review of the literature. Ultrasound Obstet Gynecol 2005;25:401– 404. 10. Matias A, Fredouille C, Nesmann C, et al: Prenatal diagnosis of left ventricular aneurysm: A report of three cases and review. Cardiol Young 1999;9:123–126.
397
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Echocardiography
Prenatal Diagnosis of Giant Left Ventricular Diverticulum: Case Report ur Gocen, M.D.,† and Selim Buyukkurt, M.D.‡ Fadli Demir, M.D.,* Nazan Ozbarlas, M.D.,* Ug *Department of Pediatric Cardiology, C ß ukurova University School of Medicine, Adana, Turkey; †Department of Cardiovascular Surgery, C ß ukurova University School of Medicine, Adana, Turkey; and ‡Department of Obstetrics and Gynecology, C ß ukurova University School of Medicine, Adana, Turkey
The distinction between a ventricular aneurysm and diverticulum is difficult. The differences between these two are based on anatomical, histological, and functional criteria. We present a case of prenatal diagnosis of a giant left ventricular outpouching at 28 weeks gestation. During the postnatal period, the neonate underwent surgical correction for the outpouching. Histopathological examination reported the resected segment was a cardiac diverticulum. Our case indicates that differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. Histopathological examinations are sometimes necessary to make a precise differentiation. Treatment should be individualized depending on the clinical presentation. (Echocardiography 2015;32:395–397) Key words: prenatal diagnosis, diverticula, fetal echocardiography, left ventricular shape
A congenital ventricular diverticulum (CVD) and aneurysm are both localized protrusions within the free wall or apex of one of the heart ventricles. Diagnosing CVD during the fetal period is very rare. CVDs usually arise from the left ventricle (LV).1,2 Although CVD etiology is not clear, an embryological developmental defect has been proposed.3 CVDs are often clinically very difficult to distinguish from a congenital aneurysm.4,5 Anatomical, histological, and functional criteria are used to differentiate between a diverticulum and an aneurysm.1,6–8 Herein, we describe a rare case of a wide-mouthed LV free wall outpouching that was identified by fetal echocardiography. Case: A 37-year-old woman (G4P3A1L3) was referred for fetal echocardiography after the detection of cardiomegaly on the comprehensive obstetric ultrasound screening. Fetal echocardiography performed at the 28th week of pregnancy showed two ventricles with normal atrioventricular and ventriculoarterial concordance. Apical four- and apical two-chamber views showed a large LV free wall outpouching (Fig. 1). The outpouching measured 30 9 14 mm in diameter; Address for correspondence and reprint requests: Fadli Demir, M.D., Department of Pediatric Cardiology, C ß ukurova University School of Medicine, 01330 Balcali, Sarıcßam, Adana, Turkey. Fax: +90 322 338 69 45; E-mail: [email protected]
the mouth of the outpouching measured 16 mm (Fig. 2). The diastolic volume of the outpouching was 5.8 mL and the ratio of the volume of the outpouching to the diastolic LV volume was 1.4. A very small pericardial effusion was seen. The thickness and acoustic properties of the outpouching wall was similar to those of the LV wall (Fig. 3). No other cardiac or extracardiac anomalies were detected. The mitral valve apparatus appeared normal. No arrhythmias or intracardiac thrombi were seen. The patient was evaluated every 4 weeks by serial echocardiograms. No changes were noted in either the size or the function of the LV and the outpouching. Over the following weeks, pericardial effusion resolved spontaneously. The baby was born at term by normal vaginal delivery and weighed 3010 g. Postnatal echocardiography showed findings similar to the fetal echocardiography: a large LV free wall outpouching with a maximum diameter of 41 9 23 mm and a wide mouth seen in Figure 4. Additionally, spontaneous echo contrast was seen in the outpouching. Measured LV ejection fraction was 55%. Outpouching showed synchrony with the interventricular septum. The patient was subsequently transferred to the neonatal intensive care unit. Upon admission, the baby had mild tachypnea; dopamine, captopril, aspirin, and low-molecular-weight heparin were started. Tachypnea and clinical findings consistent with heart failure did not subside, therefore, the Dor procedure was performed at 17 days of 395
Demir, et al.
age. During this procedure, LV aneurysmal tissue was resected with endoventricular reductoplasty, using a Dacron patch (Figs. 5 and 6). The postoperative recovery period was uneventful. A histopathological examination reported the resected segment as a cardiac diverticulum. The patient is currently 4 months old and in good general health. Conclusion: The advancement of prenatal diagnostic methods has led to prenatal early diagnosis of many rare cardiac abnormalities, such as diverticula and aneurysms. The reasons for referral for fetal echocardiography in most cases have varied from cardiomegaly to hydrops and pericardial effusion to arrhythmias seen during routine prenatal ultrasound examinations.1,2,4,6,7 Our patient was referred for cardiomegaly. The distinction between CVDs and aneurysms is important because the treatment and prognosis can differ. CVDs are synchronized with the rest of the ventricles and involve a narrow connection with the ventricular cavity. CVDs display a similar thickness and layering as the rest of the ventricular myocardium. Apical lesions have been associated with midline thoracoabdominal defects and with other cardiac malformations. Unlike CVDs, cardiac aneurysms have a broad base, appear hypokinetic, and move paradoxically to the cardiac rhythm. Congenital LV aneurysms are not typically associated with other cardiac anomalies.3,4,9 In our case, the presence of a large communication with the LV and the absence of associated cardiac and extracardiac anomalies favor the diagnosis of a ventricular aneurysm. However, synchronous contractions of the outpouching with the LV supported the diagnosis of a diverticulum. Additionally, histopathological diagnosis supported our assumption of a diverticulum.
Figure 2. Fetal echocardiogram at 32 weeks gestation. Arrow displays the left ventricular outpouching. RV = right ventricle; LV = left ventricle.
Figure 3. Intrauterine M-mode. Outpouching (black arrow) is syncronous contracting with IVS (white arrow). RV = right ventricle; LV = left ventricle; IVS = interventricular septum.
Figure 4. Postnatal echocardiography showing a markedly enlarged LV outpouching (arrow). LV = left ventricle; IVS = interventricular septum.
Figure 1. Four-chamber view fetal echocardiogram at 28 weeks gestation, showing a left ventricular outpouching (arrow).
396
The size of the CVD usually remains stable or decreases relative to the ventricle during fetal development. This natural history of the CVD is in contrast with that of a cardiac aneurysm, where enlargement throughout the pregnancy is observed.10 This increase in size is important
Giant Left Ventricular Diverticulum
A
B
Figure 5. A. Intra-operative view of the outpouching (arrow). B. Ventricular restoration using a Dacron patch (arrow).
Figure 6. Postoperative echocardiography showing a restored LV geometry and decreased LV dimension after LV diverticulum resection with endoventricular reductoplasty, using a Dacron patch (arrow). LV = left ventricle.
the outpouching demonstrated spontaneous echo contrast, which is considered an advance finding for thrombus development. In this case, the patient was symptomatic with an outpouching too large to permit proper LV function. Through the use of the Dor procedure, we expected an improvement in LV geometry and LV function. In conclusion, a fetal cardiac diverticulum and aneurysm are rare cardiac anomalies. Differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. The postnatal course of the patient may aid in the differential diagnosis; however, sometimes histopathological examination is necessary to make a precise differentiation. References
because the outcome of a fetal aneurysm is related to the ratio of volume between the aneurysm and the ventricular cavity.2–4,6 The size of the CVD in our patient remained stable throughout the pregnancy. Patients with a CVD that is not associated with other malformations are usually asymptomatic. However, some fetuses may develop an impairment of LV function, pericardial effusions, acute rupture, and fetal arrhythmias.1–3 In our case, these complications were not observed during follow-up examinations. Treatment of CVDs and aneurysms are dependent on the patient’s clinical status, presence of other cardiac abnormalities, and the size of the outpouching. Most CVD patients are asymptomatic at birth and there is a decrease in the size of the diverticulum. Patients with CVDs that are symptomatic and associated with other malformations usually require surgical intervention. The prognosis for congenital ventricular aneurysms is related to the size, course, and location of the aneurysm, degree of mitral valve involvement, associated heart failure, arrhythmias, and the presence of a thrombus.1,3,4,6 Early surgical intervention was performed in our patient since the size of the outpouching was substantial and
1. Bernasconi A, Delezoide AL, Menez F, et al: Prenatal rupture of a left ventricular diverticulum: A case report and review of the literature. Prenat Diagn 2004;24:504–507. 2. Perlitz Y, Mukary M, Lorber A, et al: Prenatal diagnosis of fetal cardiac right ventricular diverticulum disappearing at three months of age a case report and literature review. Fetal Diagn Ther 2009;25:44–46. 3. Giannoglou GD, Delioglanis SH, Paraskevaidis S, et al: Ventricular diverticulum: Definition, pathophysiology, clinical manifestations and treatment. Aristotle Univ Med J 2007;34:87–92. 4. Giacobbe L, Williams P, Ramin K, et al: Fetal diagnosis of left ventricular aneurysm: A case report. Am J Perinatol Rep 2011;1:91–94. 5. Awad SM, Patel AS, Polimenakos A, et al: Left ventricular accessory chamber: A case report and review of the literature. Pediatr Cardiol 2009;30:1022–1025. 6. Barberato MF, Barberato SH, Binotto CN, et al: Prenatal diagnosis of left ventricular aneurysm and diverticulum. Arq Bras Cardiol 2009;93:24–26. 7. Paoletti D, Robertson M: Prenatal diagnosis of a left ventricular diverticulum. AJUM 2012;15:112–114. 8. Ruiz-Esparza E, Roldan FJ, Vanquez-Antona C, et al: 2D and 3D echocardiography of left ventricular diverticulum. Echocardiography 2009;26:1087–1088. 9. McAuliffe FM, Hornberger LK, Johnson J, et al: Cardiac diverticulum with pericardial effusion: Report of two new cases treated by in-utero pericardiocentesis and a review of the literature. Ultrasound Obstet Gynecol 2005;25:401– 404. 10. Matias A, Fredouille C, Nesmann C, et al: Prenatal diagnosis of left ventricular aneurysm: A report of three cases and review. Cardiol Young 1999;9:123–126.
397
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
