© 2013, Wiley Periodicals, Inc. DOI: 10.1111/echo.12469
Echocardiography
Unruptured Sinus of Valsalva Aneurysm in a Neonate with Hypoplastic Left Heart Syndrome: Echocardiographic Diagnosis and Features Poonam Punjwani Thankavel, M.D., Matthew S. Lemler, M.D., and Claudio Ramaciotti, M.D. Department of Pediatrics, Division of Cardiology, UT Southwestern Medical Center and Children’s Medical Center of Dallas, Dallas, Texas
An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options. (Echocardiography 2014;31:E85–E87) Key words: aneurysm, congenital heart disease Case Report: A term male infant with a birth weight of 3.1 kg was delivered via C-section to a G1 woman who underwent routine prenatal care. A fetal cardiac evaluation revealed hypoplastic left heart syndrome; postnatal echocardiogram (Siemens Sequoia C-512, Siemens Medical Solutions, Mountain View, CA, USA) confirmed the diagnosis of mitral stenosis, aortic atresia, and a severely hypoplastic left ventricle (Fig. 1). In addition, an aneurysm was noted in the right ventricular outflow tract (Fig. 2) with no evidence of obstruction to flow. Color Doppler evaluation demonstrated a communication between the aneurysm and the ascending aorta with flow into the aneurysm during diastole (Fig. 3A), and into the ascending aorta during systole (Fig. 3B, movie clips S1, S2). No interventricular communication was identified, and the right ventricular systolic function was normal. Proximal coronary artery origins and course were also normal with bidirectional, unrestrictive flow noted. Due to the potential risk of rupture of the aneurysm, the patient was listed for heart transplant and bilateral pulmonary artery bands were placed at 5 days of life. Postoperative course was complicated by supraventricular tachycardia, which was managed medically. He underwent a balloon atrial septostomy at 2 weeks of age for a Address for correspondence and reprint requests: Poonam Punjwani Thankavel, M.D., Division of Pediatric Cardiology, UT Southwestern Medical Center, Children’s Medical Center of Dallas, 1935 Medical District Dr, Dallas 75235, TX, USA. Fax: (214) 456-8066; E-mail: [email protected]
restrictive interatrial communication. Following the procedure, he developed hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. He subsequently passed away at 19 days of life and the parents did not consent for an autopsy. Discussion: Sinus of Valsalva (SOV) aneurysms are rare and account for 1% of congenital cardiac anomalies.1 The majority of aneurysms originate in the right or noncoronary sinus and protrude into the right atrium or right ventricle, and are often associated with a ventricular septal defect.1 Rupture of the
Figure 1. Apical four-chamber view demonstrating the hypoplastic left ventricle. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle.
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Thankavel, et al.
Figure 2. Subcostal sagittal view demonstrating the aneurysm (asterisk) protruding into the right ventricular outflow tract (RVOT). RA = right atrium.
A
B
aneurysm usually results in congestive heart failure, leading to the diagnosis by echocardiogram. It has been previously reported as an infrequent cause of congestive heart failure in neonates.2 As unruptured aneurysms are asymptomatic, the diagnosis is often an incidental finding on echocardiogram, although rarely in adults, an unruptured aneurysm may cause obstruction to flow in the right ventricular outflow tract.3 Our case illustrates an unruptured SOV aneurysm of the right coronary sinus protruding into the right ventricle. While this finding could not be confirmed by autopsy or intraoperative assessment, the location of the aneurysm and communication with the ascending aorta are classic echocardiographic features of this lesion.4 Myocardial ischemia secondary to compression of the coronary artery by the SOV aneurysm has been reported in the literature, although usually when the left aortic sinus is involved.5Thus, evaluation of coronary anatomy and flow is imperative. The transthoracic echocardiogram on presentation showed unrestrictive flow in the right, left main, left anterior descending and circumflex coronaries, with low velocity, antegrade flow in diastole and flow into the ascending aorta during systole (movie clip S3). Potential surgical repair of the aneurysm was complicated by the diminutive size of the ascending aorta (1.5 mm). Unfortunately, the neonate had catastrophic hemodynamic instability after balloon atrial septostomy; echocardiogram at the time of ECMO support showed the SOV aneurysm was unruptured. Based on clinical events, we postulate he had an increase in systemic vascular resistance with resultant decrease in systemic cardiac output given his single ventricle physiology. To our knowledge, SOV aneurysm has not been previously reported in conjunction with hypoplastic left heart syndrome. Awareness of this constellation of findings may be helpful in its recognition and subsequent clinical management of these patients. References
Figure 3. A. Color Doppler in the parasternal long-axis plan demonstrates flow (blue) from the aneurysm into the ascending aorta during ventricular systole. B. Color Doppler in the parasternal long-axis plan demonstrates flow (arrow, red) from the ascending aorta into the aneurysm during diastole.
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1. Perloff JK: Clinical Recognition of Congenital Heart Disease, 5th ed. Philadelphia, PA: Saunders, 2003, p. 457. 2. Breviere GM, Vaksmann G, Francart C: Rupture of a sinus of Valsalva aneurysm in a neonate. Eur J Pediatr 1990;149:603–604. 3. John SH: A rare case of unruptured sinus of Valsalva aneurysm obstruction the right ventricular outflow tract. J Cardiovasc Ultrasound 2010;18:55–57. 4. Jain SP, Mahan EM, Nanda NC, et al: Doppler color flow mapping in the diagnosis of sinus of Valsalva aneurysm. Echocardiography 1989;6:533–538. 5. Lijoi A, Parodi E, Passerone GC, et al: Unruptured aneurysm of the left sinus of Valsalva causing coronary insufficiency: Case report and review of the literature. Tex Heart Inst J 2002;29:40–44.
Sinus of Valsalva Aneurysm in HLHS
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Subcostal sagittal view demonstrating the aneurysm protruding into the right ventricular outflow tract. Movie clip S2. Parasternal long-axis view with and without color Doppler demonstrating
communication of the aneurysm with the ascending aorta; flow into aorta (blue) is demonstrated during systole, and into aneurysm (red) during diastole. Movie clip S3. Parasternal short-axis view demonstrates unobstructed, bidirectional flow into the circumflex coronary artery, antegrade in diastole, retrograde in systole.
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Echocardiography
Unruptured Sinus of Valsalva Aneurysm in a Neonate with Hypoplastic Left Heart Syndrome: Echocardiographic Diagnosis and Features Poonam Punjwani Thankavel, M.D., Matthew S. Lemler, M.D., and Claudio Ramaciotti, M.D. Department of Pediatrics, Division of Cardiology, UT Southwestern Medical Center and Children’s Medical Center of Dallas, Dallas, Texas
An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options. (Echocardiography 2014;31:E85–E87) Key words: aneurysm, congenital heart disease Case Report: A term male infant with a birth weight of 3.1 kg was delivered via C-section to a G1 woman who underwent routine prenatal care. A fetal cardiac evaluation revealed hypoplastic left heart syndrome; postnatal echocardiogram (Siemens Sequoia C-512, Siemens Medical Solutions, Mountain View, CA, USA) confirmed the diagnosis of mitral stenosis, aortic atresia, and a severely hypoplastic left ventricle (Fig. 1). In addition, an aneurysm was noted in the right ventricular outflow tract (Fig. 2) with no evidence of obstruction to flow. Color Doppler evaluation demonstrated a communication between the aneurysm and the ascending aorta with flow into the aneurysm during diastole (Fig. 3A), and into the ascending aorta during systole (Fig. 3B, movie clips S1, S2). No interventricular communication was identified, and the right ventricular systolic function was normal. Proximal coronary artery origins and course were also normal with bidirectional, unrestrictive flow noted. Due to the potential risk of rupture of the aneurysm, the patient was listed for heart transplant and bilateral pulmonary artery bands were placed at 5 days of life. Postoperative course was complicated by supraventricular tachycardia, which was managed medically. He underwent a balloon atrial septostomy at 2 weeks of age for a Address for correspondence and reprint requests: Poonam Punjwani Thankavel, M.D., Division of Pediatric Cardiology, UT Southwestern Medical Center, Children’s Medical Center of Dallas, 1935 Medical District Dr, Dallas 75235, TX, USA. Fax: (214) 456-8066; E-mail: [email protected]
restrictive interatrial communication. Following the procedure, he developed hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. He subsequently passed away at 19 days of life and the parents did not consent for an autopsy. Discussion: Sinus of Valsalva (SOV) aneurysms are rare and account for 1% of congenital cardiac anomalies.1 The majority of aneurysms originate in the right or noncoronary sinus and protrude into the right atrium or right ventricle, and are often associated with a ventricular septal defect.1 Rupture of the
Figure 1. Apical four-chamber view demonstrating the hypoplastic left ventricle. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle.
E85
Thankavel, et al.
Figure 2. Subcostal sagittal view demonstrating the aneurysm (asterisk) protruding into the right ventricular outflow tract (RVOT). RA = right atrium.
A
B
aneurysm usually results in congestive heart failure, leading to the diagnosis by echocardiogram. It has been previously reported as an infrequent cause of congestive heart failure in neonates.2 As unruptured aneurysms are asymptomatic, the diagnosis is often an incidental finding on echocardiogram, although rarely in adults, an unruptured aneurysm may cause obstruction to flow in the right ventricular outflow tract.3 Our case illustrates an unruptured SOV aneurysm of the right coronary sinus protruding into the right ventricle. While this finding could not be confirmed by autopsy or intraoperative assessment, the location of the aneurysm and communication with the ascending aorta are classic echocardiographic features of this lesion.4 Myocardial ischemia secondary to compression of the coronary artery by the SOV aneurysm has been reported in the literature, although usually when the left aortic sinus is involved.5Thus, evaluation of coronary anatomy and flow is imperative. The transthoracic echocardiogram on presentation showed unrestrictive flow in the right, left main, left anterior descending and circumflex coronaries, with low velocity, antegrade flow in diastole and flow into the ascending aorta during systole (movie clip S3). Potential surgical repair of the aneurysm was complicated by the diminutive size of the ascending aorta (1.5 mm). Unfortunately, the neonate had catastrophic hemodynamic instability after balloon atrial septostomy; echocardiogram at the time of ECMO support showed the SOV aneurysm was unruptured. Based on clinical events, we postulate he had an increase in systemic vascular resistance with resultant decrease in systemic cardiac output given his single ventricle physiology. To our knowledge, SOV aneurysm has not been previously reported in conjunction with hypoplastic left heart syndrome. Awareness of this constellation of findings may be helpful in its recognition and subsequent clinical management of these patients. References
Figure 3. A. Color Doppler in the parasternal long-axis plan demonstrates flow (blue) from the aneurysm into the ascending aorta during ventricular systole. B. Color Doppler in the parasternal long-axis plan demonstrates flow (arrow, red) from the ascending aorta into the aneurysm during diastole.
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1. Perloff JK: Clinical Recognition of Congenital Heart Disease, 5th ed. Philadelphia, PA: Saunders, 2003, p. 457. 2. Breviere GM, Vaksmann G, Francart C: Rupture of a sinus of Valsalva aneurysm in a neonate. Eur J Pediatr 1990;149:603–604. 3. John SH: A rare case of unruptured sinus of Valsalva aneurysm obstruction the right ventricular outflow tract. J Cardiovasc Ultrasound 2010;18:55–57. 4. Jain SP, Mahan EM, Nanda NC, et al: Doppler color flow mapping in the diagnosis of sinus of Valsalva aneurysm. Echocardiography 1989;6:533–538. 5. Lijoi A, Parodi E, Passerone GC, et al: Unruptured aneurysm of the left sinus of Valsalva causing coronary insufficiency: Case report and review of the literature. Tex Heart Inst J 2002;29:40–44.
Sinus of Valsalva Aneurysm in HLHS
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Subcostal sagittal view demonstrating the aneurysm protruding into the right ventricular outflow tract. Movie clip S2. Parasternal long-axis view with and without color Doppler demonstrating
communication of the aneurysm with the ascending aorta; flow into aorta (blue) is demonstrated during systole, and into aneurysm (red) during diastole. Movie clip S3. Parasternal short-axis view demonstrates unobstructed, bidirectional flow into the circumflex coronary artery, antegrade in diastole, retrograde in systole.
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