So as to understand better the pathogenesis of enlargement or hypoplasia of pulmonary arteries in congenital heart disease, we studied the dimensions of the pulmonary arteries in 74 fetuses with congenital heart disease induced by administration of bis-diamine to pregnant rats. The congenital malformations induced included 12 with large ventricular septal defect, 17 with tetralogy of Fallot, 15 with tetralogy together with severe valvar pulmonary stenosis and absence of the arterial duct, 17 with tetralogy with absent pulmonary valve syndrome and absence of the arterial duct, and 13 with common arterial trunk with a confluent segment supplying the pulmonary arteries. For comparison, 16 fetuses of the same gestational age with normal hearts were studied. After rapid whole-body freezing on the 21st day of gestation, the fetuses were studied by means of serial cross-sectional photographs of the thorax. The diameter of the right pulmonary artery of the fetus was of comparable dimensions in the normal hearts (480±10 µm) (mean±SEM), those with ventricular septal defects (470±10 µm), common arterial trunk (520±20 µm), and tetralogy of Fallot (500±10 µm). These findings suggest that the commonly observed enlargement of the right pulmonary artery in patients with ventricular septal defect and common arterial trunk, and hypoplasia of the right pulmonary artery in tetralogy of Fallot, occur postnatally in response to abnormal postnatal pulmonary blood flow.

Congenital pulmonary venous stenosis is a rare but important cause of pulmonary hypertension in infancy. The prognosis is uniformly poor, despite surgery and attempted balloon dilation of the narrowed veins. We performed intraoperative stenting of congenital pulmonary venous stenoses in two infants with initial clinical and hemodynamic improvement. Both patients subsequently died due to progressive pulmonary hypertension. Stenting of the pulmonary veins in this condition does not alter the natural history.

We report a two-year-old boy with a rare combination of tetralogy of Fallot and aortic coarctation. The obstruction in this patient had an hourglass-like configuration, with a pressure difference of 15 mm Hg. There was a right aortic arch, bilateral brachiocephalic arteries, and persistent left superior caval vein. Percutaneous transluminal balloon angioplasty was performed to lower the resistance to the left ventricular ejection, considered a risk factor for subsequent corrective surgery of tetralogy. Six months later, he underwent combined repair of tetralogy of Fallot and coarctectomy with end-to-end anastomosis, thus avoiding the future potential for restenosis. During surgery, a ligament was found extending from the right subclavian artery to the distal side of the coarctation. The perioperative course was uneventful, indicating the success of the preoperative balloon angioplasty. We speculate that the embryogenesis of the coarctation could be related to an abnormal involution of the aortic arches.

An 18-month-old black child became ill one month prior to admission to our center. She was shown to be Mantoux positive, with evidence of cardiomegaly on x-ray. Echocardiography revealed a progressive enlarging left ventricular aneurysm—a finding confirmed at cardiac catheterization. She underwent successful resection of the aneurysm. Histology proved it to be tuberculous in origin. To our knowledge, this is the youngest patient reported with such an aneurysm, and the first to have survived surgical correction.

Accessory atrioventricular valvar tissue is uncommon and, on occasion, has been identified as a cause of ventricular outflow obstruction. Accessory tricuspid valvar tissue has been reported to cause subpulmonary obstruction but infrequently has accessory tissue arising from the mitral valve been associated with obstruction. This paper reports two cases of subvalvar obstruction; the first in association with a ventricular septal defect causing subaortic obstruction and the other in association with congenitally corrected transposition and a ventricular septal defect, causing subpulmonary obstruction.

A 29-year-old woman who had tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries developed aortic regurgitation due to infectious endocarditis. Isolated aortic valve replacement was undertaken because of heart failure unresponsive to medical therapy. Excessive flow through the aortopulmonary collateral arteries during cardiopulmonary bypass was controlled by means ofintraaortic balloons which were inflated to occlude the origin of the collateral arteries. Arterial return from the pump was delivered through separate cannulation of the ascending aorta and the right femoral artery. We believe that this method should be considered in those circumstances when excessive flow through systemic-to-pulmonary arteries is expected during cardiopulmonary bypass.

We report a 12-year-old boy with hypertrophic cardiomyopathy presenting with syncope. His electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block, conduction delay in the anterior fascicle of the left bundle branch, and intermittent complete atrioventricular block. We detected hypertrophic cardiomyopathy in the mother. The relationship between the diseases is discussed, and interpreted in the light of previous descriptions.

We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

A simple technique is described with which to relieve postoperative compression of the heart. Although delayed sternal closure has conventionally been used to deal with postoperative compression, this maneuver always exposes the patient to the risk of infection. We have recently employed a simple method of sternal traction when there are signs of compression on temporary sternal closure. One or two couples of sutures in the sternum are used supported by bags of saline. This simple and effective method is applicable to patients, but especially to infants and neonates, without worrying about overindications.

Balloon pulmonary valvoplasty was performed in a patient with congenitally corrected transposition, aged 28 years, who had already undergone surgical closure of a ventricular septal defect and implantation of a conduit between the pulmonary arteries and the right-sided morphologically left ventricle. The patient developed stenosis of the conduit 22 years after the procedure as confirmed by cardiac catheterization and angiocardiography, which also showed stenosis of the native pulmonary valve. A balloon valvoplasty of the native valve was carried out with improvement of the gradient from the ventricle to the pulmonary arteries. The patient developed complete heart block immediately after the procedure, which reverted to first degree block in one week and to the previous cardiac rhythm within 21 days.

A two-month-old boy, with complex pulmonary atresia, became increasingly cyanosed 10 days after a modified 5 mm right Blalock-Taussig shunt. There was no shunt flow on auscultation or Doppler echocardiography. Despite anticoagulation with heparin, the shunt remained occluded. After demonstration of the occluded shunt at angiography, a guide wire was easily passed through the occlusion and into the pulmonary artery. Balloon dilation was then successfully accomplished with a 6 mm diameter angioplasty balloon resulting in a patent shunt. The shunt was still widely patent at cardiac catheterization six months later.

Isolation of the right subclavian artery from the aortic arch is a rare malformation. When isolated, the artery originates from the right pulmonary artery via a right-sided arterial duct, which may be patent or closed at the time of presentation. Intracardiac or other anomalies of the great vessels are commonly associated with this lesion. We present a case of isolation of the right subclavian artery which was initially diagnosed as persistent patency of a left-sided arterial duct. Surgical correction was eventually achieved by disconnecting the right subclavian artery from the right pulmonary artery and translocating it to the ascending aorta.

The period of adequate palliation from a Blalock-Taussig shunt may be limited by the development of stenosis of the shunt. Balloon dilatation oftenis unsuccessful due to recoil of the vessel. We report the case of a 22-year-old man with a stenosed Blalock-Taussig shunt who was successfully treated by the implantation of a balloon expandable stent.

A symptomatic neonate was referred for aortic coarctation and, at cineangiography, was shown to have an interruption of the aortic arch at the isthmus together with coarctation related to a persistent fifth aortic arch. Balloon dilation of the coarctation was performed, but the coarctation was still evident after six months. A graft was then interposed between the left subclavian artery and the descending aorta. Balloon dilation was repeated five years later leaving a residual gradient of 11 mm Hg.

A case is presented of a 3.5-year-old boy with aorto-left ventricular tunnel. A diagnosis of isolated aortic regurgitation had been made at the age of 11 months following cardiac catheterization with selective left ventricular angiography and aortography. The correct diagnosis was established 2.5 years later following cross-sectional echocardiographic examination combined with color Doppler investigation.

Aneurysmal dilatation is a rare malformation of the arterial duct. Successful repair of this anomaly was achieved in a five-day-old neonate, using deep hypothermia and circulatory arrest. Of the cases of aneurysm of the arterial duct reported in the English literature, only seven have been treated surgically with success.

A 43-year-old female with complaints of exertional dyspnea was found by Doppler echocardiography to have fixed subaortic stenosis and a redundant mitral valve which, during systole, formed a chamber in the left ventricular outflow tract. This resulted in a subaortic gradient of 40 mm Hg. There was also moderate aortic regurgitation.

A rare association of ‘absent pulmonary valve’ syndrome, midmuscular ventricular septal defect and aneurysmatic dilation of the ascending aorta without pathology of the aortic valve is described in a 10-month-old infant. The diagnosis was established by echocardiography and angiography.

A homogeneous opacity of the right upper lobe was found radiographically in a 15-month-old male child during investigation of an upper respiratory tract infection. Based on computerized tomography, the diagnosis was made of a solid tumor of the lung. At thoracotomy, dilated serpentine vessels were found on the surface of the right upper lobe, and the diagnosis of a large arteriovenous malformation was considered. Use of enhanced computed tomography without contrast and failure to note a reduced arterial oxygen saturation were two diagnostic pitfalls. The diagnosis of an arteriovenous malformation was confirmed by pulmonary angiography, and a right upper lobectomy was successfully performed.

Summary Absence of the arterial duct has been reported in association with tetralogy of Fallot, with pulmonary stenosis or atresia, other complex cyanotic congenital heart diseases, and anomalies of the aortic arch but not, as far as we are aware, with tricuspid atresia. We report the clinical, surgical, and autopsy findings of a patient with tricuspid atresia whose initial clinical course was influenced by the absence of thearterial duct.