Postoperatively, both patients ex- ers, MD, for his assistance in the hibited labile pulmonary hyperten- preparation of the manuscript and sion. In our current experience of also to James Joransen, MD, and over 200 patients with TGA and in- Paul Woolf, MD, for their assistance tact ventricular septum who have in providing the follow-up data on undergone an arterial switch proce- these patients. dure between 1983 and 1990, only these 2 patients manifested suprasystemic pulmonary artery pressure with hemodynamic compromise in 1. Dick M, Heidelberger K, Crowley D. Quantitative analysis of the pulmonary arteries in the immediate postoperative period. morphometric two patients with D-transposition of the great arterAlthough these episodes of puhno- ies and persistence of fetal circulation. Pediatr Rex nary hypertension were often severe, 1981;15:1397~1401. RE, Freedom RM, Rowe RD. Persisthey were successfully managed with 2.tenceHawker of the fetal pattern of circulation in transposianesthesia, hyperventilation and ino- tion of the great arteries. Hopkins Med 1974;134: tropic support. Last, placement of a 107-l 17. 3. Rudolph AM, Yuan S. Response of the pulmotransthoracic pulmonary artery nary vasculature to hypoxia and H+ ion concentracatheter5 at surgery allows prompt tion changes. J Clin Inuest 1966;45:399-404. 4. Lang P, Freed MD, Bierman FZ. Use of prostarecognition and treatment of pulmoglandin El in infants with d-transposition of the great nary hypertensive crises. arteries and intact ventricular septum. Am J Cardiol
septotomy established an adequatesized intercirculatory communication, and efforts to increase 02 delivery (by use of inotropic agents to augment cardiac output) and to decrease 02 consumption (by narcotic anesthesia and neuromuscular blockade) were made, intermittent episodes of severe hypoxemia and cardiac decompensation persisted in both of our neonates. Additional management strategies included decreasing pulmonary vascular resistance by alkalinization, achieved either by hyperventilation or bicarbonate infusion.3 Prostaglandin infusion can also be used to lower pulmonary vascular resistance, and has been shown to increase oxygenation in patients with TGA and adequate atria1 communication who remained critically cyanotic after an atria1 septotomy.4
wish to express our gratitude to Stephen SandAcknowledgment:
We
Rupture of Congenital Sinus of Valsalva Aneurysm in a Newborn Lowell W. Perry, MD, Gerard R. Martin, and Frank M. Midgley, MD
upture of a sinus of Valsalva aneurysm is unusual, gener. ally occurring in the second to fourth decades of life1m5 with the youngest previously reported patient aged 10 months.6 The present report describes the lesion in a newborn infant.
R
The patient, a 4.7 kg, 8-day-old girl, was referred because of a short, grade 216 systolic precordial murmur, a grade 4 pandiastolic murmur with a thrill over the lower left sternal border, and heart failure. Echocardiography demonstrated enlargement of the aortic root and aneurysm of the right siFrom the Departments of Pediatric Cardiology and Cardiovascular Surgery, Children’s National Medical Center, George Washington University School of Medicine and Georgetown University Children’s Medical Center, 3800 Reservoir Road, NW, Washington, D.C. 20007.
MD,
Frank M. Galioto, Jr.,
MD,
nus of Valsalva with rupture into the right ventricular outflow tract (Figure 1). The coronary arteries were normal. Electrocardiogram revealed right ventricular hypertrophy with a normal QRS axis. Cardiomegaly and increased pulmonary flow were present on chest roentgenogram. Cardiac catheterization at 8 days of age demonstrated a left-to-right shunt at atria1 and pulmonary artery levels with a Qp/Qs of 2.9, systemicpressure in the pulmonary artery and right ventricle, and pulmonary vascular resistance of 6.4 Wood units. Cineangiography demonstrated herniation of the right coronary sinus into the right ventricular outflow tract. The ductus arteriosus was closed. Anomalous origin of the right subclavian artery from descending aorta and
1979:44:76-S 1. 5. Gold JP, Jonas RA, Lang P. Transthoracic intracardiac monitoring lines in pediatric patients: a ten year experience. Ann ThoracSurg 1986;42:185-195.
atria1 left-to-right shunt also were present on cineangiography. There was no aortic valve insufficiency. During open heart surgery at 14 days of age, cardiomegaly and a discrete aneurysmal dilatation of the infundibulum chamber were noted. Inspection of the subpulmonary chamber by way of a main pulmonary artery incision demonstrated an aneurysm of the right sinus of Valsalva with communication to the infundibulum well inferior to the pulmonary valve. The apex of the aneurysm had multiple holes and aortic valve tissue was not involved. The aneurysm was repaired with a Dacron@ velour patch. The ductus arteriosus had reopened and was ligated. The atria1 septal defect was closed with a Dacron velour patch. Right heart pressure decreased to one third of systemic pressure on direct measurement after cardiopulmonary bypass. The patient was discharged on the seventh postoperative day.
Congenital aneurysm of the sinus of Valsalva has not been reported CASE REPORTS
1255
FIGURE 1. Left, short-axis view dcmomtrating
aneurysm (arrow) from right caronary Row bpplsr echocadiam showing flow from aorta to right ventricular AO=aorta;L=left;LA=leff~P=~PA=plllmonaryartery;R=right;RA=rightatrium;RCA=rigMcoronary
Right, cdor
sinus to rigM outfk~w tract
ventridar (arrqw).
outflow tract. A = anterior;
artery.
previously as producing signs and symptoms in the newborn period, Congenital aneurysms involve the right sinus in three fourths and the noncoronary sinus in one fourth of cases. Aneurysms of the left coronary sinus are unusual. Aneurysms of the noncoronary sinus rupture into the right atrium. Aneurysms of the right coronary sinus rupture into the right atrium, right ventricular inflow or outflow tract beneath the ptdmonary valve, the latter being associated with a ventricular septal defect in approximately half of reported cases.l The presence of a diastolic thrill in a newborn infant raises the clinical differential diagnosis of tetralogy of Fallot with absent pulmonary valve, congenital mitral stenosis and aort&-left ventricular tunnel, which are easily excluded by echocardio-
1256
THE AMERICAN
JOURNAL
graphic evaluation. Supracristal ventricular septal defect with herniation of the aortic valve was excluded by catheterization. Coronary artery fistulas were excluded by echocardiography and cineangiography. With rupture of a sinus of Valsalva aneurysm into the right atrium, flail tricuspid valve, right atria1 myxoma, tricuspid valve vegetations and pedunculated right atria1 thrombus also must be excluded.3 There was nothing in our patient to suggest bacterial endocarditis.4 Ruptured congenital aneurysms of the sinus of Valsalva may present with murmurs and heart failure in the newborn period. The diagnosis can be suspected by echocardiographic evaluation and confirmed by catheterization, which is required to exclude associated anomalies.3 Early surgical repair results in rapid return
OF CARDIOLOGY
VOLUME
68
NOVEMBER
1, 1991
of cardiac function to normal.5 Delayed surgical repair is associated with significant morbidity and mortality.6 1. Terdjman M, Bourdarias J, Farcot J, Gueret P, Duburg 0, Ferrier A, Hanania G. Aneurysms of sinus of Valsalva: two-dimensional echocardiographic diagnosis and recognition of rupture into right heart cavities. JAm CollCardiol1984;3:1277-1235. 2. Mayer ED, Ruffman K, Saggu W, Butzmann B, Bernhardt-Mayer K, Shatton N, Schmitz W. Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg 1986;42:8 1-85. 3. Tonkin IL, Maria-Garica J, Paul RN, Bell ED Jr, Ilabaca P. Ruptured sinus of Valsalva aneurysm in children: report of 2 cases and literature review. Cardiouasc Intervent Radio1 1984;7:78-83. 4. Shaffer EM, Snider AR, Beckman RH, Behrendt DM, Peschiera AW. Sinus of Valsalva aneurysm complicating bacterial endocarditis in infants: diagnosis with two-dimensional and Doppler echocardiography. J Am Co11 Cardiol 1987;9:588-591. 5. Bantos P, Sousa A, Cunah D, Games MR. Congenital aneurysm of the sinus of Valsalva. Thorac Cardiovasc Surg 1985;33:125-127. 6. Burakovsky VI, Pcdsolkov VP, Sabirow BN, Nasedkina MA, Alekian BG, Dvinyaninova NB. Ruptured congenital aneurysm of the sinus of Valsalva. J Thorac Cardiovasc Surg 1988;95:836-841.
septotomy established an adequatesized intercirculatory communication, and efforts to increase 02 delivery (by use of inotropic agents to augment cardiac output) and to decrease 02 consumption (by narcotic anesthesia and neuromuscular blockade) were made, intermittent episodes of severe hypoxemia and cardiac decompensation persisted in both of our neonates. Additional management strategies included decreasing pulmonary vascular resistance by alkalinization, achieved either by hyperventilation or bicarbonate infusion.3 Prostaglandin infusion can also be used to lower pulmonary vascular resistance, and has been shown to increase oxygenation in patients with TGA and adequate atria1 communication who remained critically cyanotic after an atria1 septotomy.4
wish to express our gratitude to Stephen SandAcknowledgment:
We
Rupture of Congenital Sinus of Valsalva Aneurysm in a Newborn Lowell W. Perry, MD, Gerard R. Martin, and Frank M. Midgley, MD
upture of a sinus of Valsalva aneurysm is unusual, gener. ally occurring in the second to fourth decades of life1m5 with the youngest previously reported patient aged 10 months.6 The present report describes the lesion in a newborn infant.
R
The patient, a 4.7 kg, 8-day-old girl, was referred because of a short, grade 216 systolic precordial murmur, a grade 4 pandiastolic murmur with a thrill over the lower left sternal border, and heart failure. Echocardiography demonstrated enlargement of the aortic root and aneurysm of the right siFrom the Departments of Pediatric Cardiology and Cardiovascular Surgery, Children’s National Medical Center, George Washington University School of Medicine and Georgetown University Children’s Medical Center, 3800 Reservoir Road, NW, Washington, D.C. 20007.
MD,
Frank M. Galioto, Jr.,
MD,
nus of Valsalva with rupture into the right ventricular outflow tract (Figure 1). The coronary arteries were normal. Electrocardiogram revealed right ventricular hypertrophy with a normal QRS axis. Cardiomegaly and increased pulmonary flow were present on chest roentgenogram. Cardiac catheterization at 8 days of age demonstrated a left-to-right shunt at atria1 and pulmonary artery levels with a Qp/Qs of 2.9, systemicpressure in the pulmonary artery and right ventricle, and pulmonary vascular resistance of 6.4 Wood units. Cineangiography demonstrated herniation of the right coronary sinus into the right ventricular outflow tract. The ductus arteriosus was closed. Anomalous origin of the right subclavian artery from descending aorta and
1979:44:76-S 1. 5. Gold JP, Jonas RA, Lang P. Transthoracic intracardiac monitoring lines in pediatric patients: a ten year experience. Ann ThoracSurg 1986;42:185-195.
atria1 left-to-right shunt also were present on cineangiography. There was no aortic valve insufficiency. During open heart surgery at 14 days of age, cardiomegaly and a discrete aneurysmal dilatation of the infundibulum chamber were noted. Inspection of the subpulmonary chamber by way of a main pulmonary artery incision demonstrated an aneurysm of the right sinus of Valsalva with communication to the infundibulum well inferior to the pulmonary valve. The apex of the aneurysm had multiple holes and aortic valve tissue was not involved. The aneurysm was repaired with a Dacron@ velour patch. The ductus arteriosus had reopened and was ligated. The atria1 septal defect was closed with a Dacron velour patch. Right heart pressure decreased to one third of systemic pressure on direct measurement after cardiopulmonary bypass. The patient was discharged on the seventh postoperative day.
Congenital aneurysm of the sinus of Valsalva has not been reported CASE REPORTS
1255
FIGURE 1. Left, short-axis view dcmomtrating
aneurysm (arrow) from right caronary Row bpplsr echocadiam showing flow from aorta to right ventricular AO=aorta;L=left;LA=leff~P=~PA=plllmonaryartery;R=right;RA=rightatrium;RCA=rigMcoronary
Right, cdor
sinus to rigM outfk~w tract
ventridar (arrqw).
outflow tract. A = anterior;
artery.
previously as producing signs and symptoms in the newborn period, Congenital aneurysms involve the right sinus in three fourths and the noncoronary sinus in one fourth of cases. Aneurysms of the left coronary sinus are unusual. Aneurysms of the noncoronary sinus rupture into the right atrium. Aneurysms of the right coronary sinus rupture into the right atrium, right ventricular inflow or outflow tract beneath the ptdmonary valve, the latter being associated with a ventricular septal defect in approximately half of reported cases.l The presence of a diastolic thrill in a newborn infant raises the clinical differential diagnosis of tetralogy of Fallot with absent pulmonary valve, congenital mitral stenosis and aort&-left ventricular tunnel, which are easily excluded by echocardio-
1256
THE AMERICAN
JOURNAL
graphic evaluation. Supracristal ventricular septal defect with herniation of the aortic valve was excluded by catheterization. Coronary artery fistulas were excluded by echocardiography and cineangiography. With rupture of a sinus of Valsalva aneurysm into the right atrium, flail tricuspid valve, right atria1 myxoma, tricuspid valve vegetations and pedunculated right atria1 thrombus also must be excluded.3 There was nothing in our patient to suggest bacterial endocarditis.4 Ruptured congenital aneurysms of the sinus of Valsalva may present with murmurs and heart failure in the newborn period. The diagnosis can be suspected by echocardiographic evaluation and confirmed by catheterization, which is required to exclude associated anomalies.3 Early surgical repair results in rapid return
OF CARDIOLOGY
VOLUME
68
NOVEMBER
1, 1991
of cardiac function to normal.5 Delayed surgical repair is associated with significant morbidity and mortality.6 1. Terdjman M, Bourdarias J, Farcot J, Gueret P, Duburg 0, Ferrier A, Hanania G. Aneurysms of sinus of Valsalva: two-dimensional echocardiographic diagnosis and recognition of rupture into right heart cavities. JAm CollCardiol1984;3:1277-1235. 2. Mayer ED, Ruffman K, Saggu W, Butzmann B, Bernhardt-Mayer K, Shatton N, Schmitz W. Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg 1986;42:8 1-85. 3. Tonkin IL, Maria-Garica J, Paul RN, Bell ED Jr, Ilabaca P. Ruptured sinus of Valsalva aneurysm in children: report of 2 cases and literature review. Cardiouasc Intervent Radio1 1984;7:78-83. 4. Shaffer EM, Snider AR, Beckman RH, Behrendt DM, Peschiera AW. Sinus of Valsalva aneurysm complicating bacterial endocarditis in infants: diagnosis with two-dimensional and Doppler echocardiography. J Am Co11 Cardiol 1987;9:588-591. 5. Bantos P, Sousa A, Cunah D, Games MR. Congenital aneurysm of the sinus of Valsalva. Thorac Cardiovasc Surg 1985;33:125-127. 6. Burakovsky VI, Pcdsolkov VP, Sabirow BN, Nasedkina MA, Alekian BG, Dvinyaninova NB. Ruptured congenital aneurysm of the sinus of Valsalva. J Thorac Cardiovasc Surg 1988;95:836-841.
