[Pulmonary Artery Replacement in a Patient with Pulmonary Artery Dissection Associated with Pulmonary Hypertension and Single Ventricle:Report of a Case].

A right-sided classical Blalock-Taussig shunt was created in a 3-year-old male infant with hypoplastic left ventricle, common atrioventricular valve, transposition of the great arteries, and pulmonary stenosis. He was diagnosed with near Eisenmenger pulmonary hypertension at the age of 6 years. Surgery to achieve right heart bypass circulation was not conducted. At 33 years of age, he experienced hemoptysis. Pulmonary artery dissection, a life-threatening condition, was detected upon computed tomography performed when the patient was 35 years of age. After three weeks, surgery with cardiopulmonary bypass requiring pulmonary artery replacement using composite prosthetic conduits, closure of the pulmonary valve, and banding of the previously created Blalock-Taussig shunt was performed. Six years after the surgery, the patient's physical activity is maintained. Although intracardiac repair or definitive palliation with cardiopulmonary bypass is contraindicated in such patients, life-saving surgery with cardiopulmonary bypass that does not worsen pulmonary hypertension can be performed.

[Spontaneous Closure of Atrial Septal Defect Caused Severe Heart Failure in a Child with Pulmonary Atresia and Intact Ventricular Septum Having an Extracardiac Total Cavopulmonary Connection;Report of a Case].

Unobstructed atrial communication is a prerequisite for favorable pulmonary circulation in patients with single-ventricle physiology and atrial-communication-dependent pulmonary venous drainage. It might also be a prerequisite for favorable coronary circulation in patients with single-ventricle physiology and atrial-communication-dependent coronary venous drainage. Obstruction of atrial communication, on which coronary venous drainage depends, has not yet been documented. We report a patient with pulmonary atresia and an intact ventricular septum having an extracardiac total cavopulmonary connection. Spontaneous closure of the atrial septal defect( ASD), measuring 10 mm before the total cavopulmonary connection, led to a leftward ventricular septal shift and left ventricular wall thickening, causing severe left ventricular diastolic dysfunction which resolved after surgical ASD creation. Detection of spontaneous ASD closure by ultrasound was challenging because the to-and-fro blood flow in the dilated coronary sinus mimicked a patent ASD. Prophylactic ASD enlargement accompanying the cavopulmonary connection procedure is advised in such patients.

[Total Repair of Pulmonary Atresia, Ventricular Septal Defect, and Major Aorto-pulmonary Collateral Arteries That were Deemed Unrepairable in an Adolescent and Adults].

Pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries are deemed unrepairable in some patients. Reportedly, 39% to 63% of patients treated by the staged unifocalization pathway, and 25% to 51% of patients treated by the pulmonary artery rehabilitation strategy were deemed unsuitable to undergo total repair. Single-stage complete unifocalization with intracardiac repair can be performed in some older patients with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable. We report four such patients with ages between 12 years and 28 years at surgery. Bosentan hydrate was administered pre-operatively in one female patient for 14 months to ameliorate regional pulmonary hypertension. Seventeen months after repair, she delivered a baby by cesarean section. One patient underwent aortic valve replacement concomitant with repair due to severe aortic regurgitation caused by healed infective endocarditis. All patients were discharged home 9 to 18 days after surgery. After 72 to 100 months of follow-up, three patients are in New York Heart Association (NYHA) class Ⅰ, and the remaining patient are in NYHA class Ⅱ. Single-stage complete unifocalization can allow total repair in adolescents and adults with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable.