Scimitar syndrome with left pulmonary hypertension and right pulmonary artery stenosis in childhood--report of a case of successful surgical repair

An one-year old child with Scimitar syndrome (anomalous drainage of the right pulmonary vein to inferior vena cava with right lung hypoplasia) associated with left side pulmonary hypertension and right pulmonary artery stenosis underwent surgical repair. The anomalous pulmonary vein was anastomosed to right atrium with 10 mm PTFE graft interposition and drained to left atrium through equine pericardial intra-atrial baffle. The stenotic portion of right pulmonary artery was enlarged with porcine pericardial patch. To our knowledge, this is the first successful surgical case of Scimitar syndrome with pulmonary hypertension in childhood reported in literature. A repair of anomalous pulmonary venous drainage to inferior vena cava in childhood is difficult, and a meticulous selection of surgical procedure is necessary.     

Hypoplastic left heart syndrome with anomalous origin of the right coronary artery

Hypoplastic left heart syndrome in association with an anomalous origin of a coronary artery from the pulmonary artery is a very rare congenital malformation. In the few reported cases, the left coronary artery or the circumflex artery arises from the right pulmonary artery. We describe a newborn who presented with hypoplastic left heart syndrome, and at the time of operation had an anomalous origin of the right coronary artery from the right pulmonary artery that was detected. The patient underwent a successful modified Norwood procedure with direct reimplantation of the right coronary artery to the neo-aorta.     

Anomalous left anterior descending coronary artery from pulmonary artery with ventricular septal defect

Anomalous origin of the left anterior descending coronary artery with associated congenital defects is very rare. An angiogram of a 47-year-old woman admitted for a ventricular septal defect closure revealed an anomalous left anterior descending coronary artery arising from the left posterior sinus of the pulmonary artery. During the surgical procedure, the origin of the left anterior descending coronary artery was closed with pledgetted polypropylene sutures through the pulmonary artery. The ventricular septal defect was closed with a patch through the right atrium, and the left anterior descending coronary artery was bypassed with the left internal mammary artery.     

Anomalous pulmonary artery from the aorta via a patent ductus arteriosus: repair in a premature infant

A successful repair of anomalous left pulmonary artery from the ascending aorta via a left ductus arteriosus in a 1 kg baby is reported. Repair was performed at an early age to avoid pulmonary hypertension and left pulmonary artery occlusion. Utilizing the right ductus to perfuse the right lung, surgery was performed without cardiopulmonary bypass.     

A case of reoperation 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery

We experienced a case of a 38-year-old woman with a persistent cough, 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery. The right pulmonary artery was massively dilated, thus it caused the compression of the bronchi, whitch was thought to result in her respiratory symptom. This dilatation of the right pulmonary artery seemed to have progressed because of the following two reasons. The first is the pulmonary hypertension caused by the late reconstruction of the left pulmonary artery. The second is residual pulmonary stenosis and regurgitation after the initial operation without a pulmonary valve insertion. We performed a reoperation consisting of reconstruction of the right ventricular outflow tract using a valved conduit and plication of the right pulmonary artery. Her postoperative course has been without any complications and satisfactory for the past 2 years.     

A case of reoperation 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery]

We experienced a case of a 38-year-old woman with a persistent cough, 24 years after repair of absent pulmonary valve syndrome with anomalous origin of the left pulmonary artery. The right pulmonary artery was massively dilated, thus it caused the compression of the bronchi, which was thought to result in her respiratory symptom. This dilatation of the right pulmonary artery seemed to have progressed because of the following two reasons. The first is the pulmonary hypertension caused by the late reconstruction of the left pulmonary artery. The second is residual pulmonary stenosis and regurgitation after the initial operation without a pulmonary valve insertion. We performed a reoperation consisting of reconstruction of the right ventricular outflow tract using a valved conduit and plication of the right pulmonary artery. Her postoperative course has been without any complications and satisfactory for the past 2 years.     

Anomalous origin of the left coronary artery from the pulmonary artery: new electrocardiographic, echocardiographic and surgical observations

The case of a two year old girl with anomalous origin of the left coronary artery from the pulmonary artery is described. She was never in heart failure but had cardiomegaly and anginal pain. The ECG showed a typical infarct pattern with left ventricular hypertrophy. An unusual finding was a prolonged QTc of 0.52. During cardiac catheterization and twice 24 hours later she developed ventricular fibrillation treated with electroshock and prevented later with propranolol. The QTc returned to normal after surgery. Echocardiography showed diastolic flutter and early systolic closure of the pulmonary valve. This disappeared after surgical correction. Transverse 2D echo of the aortic root showed a large right coronary artery which decreased in size after surgery. The left coronary artery was not seen on echocardiography. At cardiac catheterization the diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery was established, with a large shunt to the pulmonary artery through the anomalous artery. Mild pulmonary hypertension and mild mitral regurgitation were present. At surgery, since direct implantation was technically impossible, the left coronary artery was successfully connected to the aorta via a 6 mm expanded Poly-Tetra-Fluoro-Ethylene (P.T.F.E.) graft.     

Successful mitral valve replacement and myocardial revascularization in an adult with anomalous origin of the left coronary artery from the pulmonary artery

A 35-year-old white woman with anomalous origin of the left coronary artery from the pulmonary artery had infective endocarditis and severe mitral regurgitation. She survived mitral valve replacement and occlusion of the anomalous left main stem, but subsequently deterioration of her condition necessitated urgent saphenous vein bypass grafts to the left anterior descending and circumflex coronary arteries. Pulmonary hypertension with raised right ventricular end-diastolic pressure probably compromised collateral flow from the right to the left coronary systems.     

Unilateral pulmonary oedema in a patient with hypertrophic cardiomyopathy and severe narrowing of the left pulmonary artery.

A patient with hypertrophic cardiomyopathy developed unilateral right sided pulmonary oedema. Pulmonary angiography showed a proximal severe narrowing of the left pulmonary artery.     

Successful surgical correction of anomalous origin of the right pulmonary artery from the aorta in an adult

We report an adult patient with anomalous right pulmonary artery (RPA) from the ascending aorta with origin stenosis, a secundum type of atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) in the left lung, and a protected right lung. Restoration of the continuity between the RPA and the left pulmonary arterial system was achieved without cardiopulmonary bypass.     

Anomalous origin of the right pulmonary artery from the ascending aorta: a report of three cases

Three patients with anomalous origin of the right pulmonary artery from the ascending aorta were reported. Case 1 was a 16-day-old girl with the right pulmonary artery arising from the base of the ascending aorta. Division and direct anastomosis of the anomalous vessel to the pulmonary trunk were successfully performed. Case 2 was a 4-month-old girl with the kinked and stenotic right pulmonary artery arising from the distal ascending aorta. At the time of surgery, however, the right pulmonary artery had been completely occluded and the pulmonary hypertension of the main pulmonary trunk had been disappeared. The chest was closed without any corrections. Case 3 was a 17-month-old girl with the stenotic right pulmonary artery arising from the distal ascending aorta. The stenotic lesion of the right pulmonary artery was longitudinally incised and anastomosed with the PTFE graft in an end-to-end fashion, followed by the end-to-side anastomosis of the graft and the pulmonary trunk.     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.     

Anomalous origin of the right pulmonary artery from the ascending aorta associated with aortopulmonary window.

A rare case of anomalous origin of the right pulmonary artery from the ascending aorta associated with an aortopulmonary window and severe pulmonary hypertension in a 13-year-old girl is reported. The window was closed using a Dacron patch and the continuity between the anomalous vessel and the main pulmonary artery was restored with a tubular Dacron graft. After operation the pulmonary pressure dropped to about one-half of the systemic pressure, and equal perfusion and ventilation of both lungs was demonstrated. Although early recognition and surgical treatment of this condition are mandatory to prevent the onset of irreversible pulmonary vascular disease, the presence of severe pulmonary hypertension did not contraindicate surgical correction in our patient.     

Anomalous origin of the pulmonary artery from the right coronary artery

A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.     

Anomalous left coronary artery from pulmonary artery with mitral stenosis

The usual presentation of anomalous left coronary artery from pulmonary artery is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. The manifestations of left heart failure may be masked if pulmonary artery pressure remains high. We believe this is a rarest of rare case of anomalous left coronary artery from pulmonary artery with severe mitral stenosis and pulmonary hypertension in which pulmonary hypertension, along with good collateral circulation helped to preserve left ventricular function.     

Repair of tetralogy of Fallot associated with anomalous origin of right pulmonary artery and accessory left anterior descending coronary artery

We present an unusual combination of lesions in an 8-month-old child diagnosed with tetralogy of Fallot, anomalous origin of right pulmonary artery, and anomalous coronary artery crossing the pulmonary annulus. The association of anomalous origin of branch pulmonary artery from aorta and tetralogy of Fallot (TOF) is extremely rare with an incidence of 0.4%. The incidence of anomalous coronary artery in TOF is 10.3%. However, a combination of all three lesions poses challenges to surgical repair and has not been previously reported.     

Surgical correction of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta

A surgically corrected case of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta is presented. The patient was under-developed 3-year-old girl. She weighed only 1,000 grams at birth and had a systolic heart murmur and slight cyanosis. Echocardiography demonstrated the features of tetralogy of Fallot shortly after birth. Chest roentgenogram showed right ventricular enlargement, but vascular marking of lung was almost normal. Catheterization and cineangiography demonstrated the findings of tetralogy of Fallot. Final diagnosis of anomalous origin of left pulmonary artery from ascending aorta was confirmed by aortography. Surgical correction was accomplished using cardiopulmonary bypass with surface cooling. The left pulmonary artery was detached from the aorta and anastomosed to the main pulmonary artery. Pulmonary valve stenosis and severe infundibular hypertrophy were corrected, and ventricular septal defect was closed with a patch. Postoperative recovery was uneventful. On follow up after surgery, she appears to be in very good condition.     

An alternative procedure for correction of anomalous origin of left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that is commonly associated with mitral insufficiency. Direct implantation of the anomalous origin of the left coronary artery from the pulmonary artery into the aorta is ideal, but it may not be fit for some patients whose anomalous left coronary arteries arise remotely from the ascending aorta. To solve the same technical problem in a boy with anomalous origin of the left coronary artery from the pulmonary artery in combination with moderate mitral insufficiency, we successfully elongated the anomalous coronary artery by creating a tube-shape graft using part of the pulmonary arterial wall. Simultaneous mitral annuloplasty was performed after a dual-coronary repair.     

Surgical repair for pulmonary artery sling on a 46-day-old infant

Surgical repair on a 46-day-old girl with pulmonary artery sling is reported. She was suffered from dypnea and admitted to a hospital on 12 days after her birth. On chest roentgenogram atelectasis of right lung was found. She had been on respirator since 21 days after her birth. On bronchogram and pulmonary arteriogram, the trachea and right bronchus were compressed and shifted with the anomalous origin of left pulmonary artery which originated from the right pulmonary artery and passed between the trachea and esophagus. These results confirmed the diagnosis of pulmonary artery sling. Hence, she was referred to our hospital for surgical treatment. She underwent surgical repair on 46-day-old. In operation, we chose a mid-sternal splitting incision, and excised 5 mm of ductus arteriosus. Under extracorporeal circulation, the left pulmonary artery was amptated from the right pulmonary artery and pulled back to left side between the trachea and the esophagus. The left pulmonary artery was anastomosed to the main pulmonary artery at the anterior to the left bronchus. She weaned from respirator, and was extubated on the 3rd day after procedure. She recovered uneventfully in post-operative course. On the 24th day after operation she discharged from hospital. On pulmonary perfusion scanning and pulmonary arteriography performed one year after operation, the left pulmonary artery was patient with slightly decreased perfusion in the left lung. Surgical repair for pulmonary artery sling was recognized as high mortality because of frequently associated tracheobronchial anomalies. In the Japanese literature, only 4 patients survived surgically and lived in late stage.(ABSTRACT TRUNCATED AT 250 WORDS)