[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.

[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.

[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.

Successful Truncal Valve Replacement With a Mechanical Valve After Bilateral Pulmonary Artery Banding.

Surgical management of truncus arteriosus, or common arterial trunk, has expanded over the past three decades to include earlier surgical intervention, initially during infancy and now chiefly in the neonatal period. Many studies have shown that preoperative truncal valve insufficiency is an independent risk factor for mortality. We herein present the case of a five-month-old boy with severe truncal valve insufficiency who underwent repair of common arterial trunk and prosthetic replacement of the truncal valve with associated annular enlargement after initial stabilization in the newborn period via palliative bilateral pulmonary artery banding. We believe that initial bilateral pulmonary artery banding represents a viable option for some cases of common arterial trunk involving truncal valve insufficiency.

[Bilateral pulmonary artery banding using ligation clips and facile Norwood-Glenn procedure].

Bilateral pulmonary artery banding( BPAB), though a less-invasive surgical option for hypoplastic left heart syndrome (HLHS), entails considerable risk of residual pulmonary artery stenosis after de-banding. Autologous aortic reconstruction in Norwood procedure is attractive in terms of growth potential, but technically demanding. To overcome these drawbacks, we modified the 2 techniques. Eight patients with HLHS underwent BPAB whereby ligation clips were half-closed into rhombic shape to deform bilateral pulmonary arteries. The arterial duct was kept patent by prostaglandin E1 infusion. One patient died of sepsis( age 8 months), while the 7 survivors underwent Norwood-Glenn procedure. Both pulmonary arteries were excised from the pulmonary trunk with minimal cuffs. Resultant defect in the pulmonary trunk was longitudinally closed. After arterial duct excision, pulmonary trunk-to descending aorta continuity was reconstructed by end-to-end anastomosis. Ascending aorta-to-aortic arch complex was anastomosed to the pulmonary trunk in a side-to-side fashion. After bilateral pulmonary artery continuity was reconstructed, Glenn anastomosis was made. One patient died of pneumonia(age 5 months). Currently, the 6 surviving patients(age 4∼30 months), enjoy good health. Four of them have completed Fontan procedure. Our modified techniques are facile, reproducible, and pose low risk of residual pulmonary artery stenosis or aortic stenosis.

Adhesion barrier reduces postoperative adhesions after cardiac surgery.

Reoperation in cardiac surgery is associated with increased risk due to surgical adhesions. Application of a bioresorbable material could theoretically reduce adhesions and allow later development of a free dissection plane for cardiac reoperation. Twenty-one patients in whom a bioresorbable hyaluronic acid-carboxymethylcellulose adhesion barrier had been applied in a preceding surgery underwent reoperations, while 23 patients underwent reoperations during the same period without a prior adhesion barrier. Blinded observers graded the tenacity of the adhesions from surgical video recordings of the reoperations. No excessive bleeding requiring wound reexploration, mediastinal infection, or other complication attributable to the adhesion barrier occurred. Multiple regression analysis showed that shorter duration of the preceding surgery, non-use of cardiopulmonary bypass in the preceding surgery, and use of the adhesion barrier were significantly associated with less tenacious surgical adhesions. The use of a bioresorbable material in cardiac surgery reduced postoperative adhesions, facilitated reoperation, and did not promote complications. The use of adhesion barrier is recommended in planned staged procedures and those in which future reoperation is likely.

[New technique of bilateral pulmonary artery banding using half-closed surgical clips; an experimental study].

Bilateral pulmonary artery banding (PAB) using constrictive tapes encircled around both pulmonary arteries entails frequent post-debanding residual pulmonary artery stenosis. We devised a new technique of bilateral PAB using half-closed surgical clips. Surgical clips, applied half-closed around both pulmonary arteries, transform the cross sections of the pulmonary arteries into diamond-shapes, and reduce their cross sectional areas without significantly reducing their circumferences. Animal experiment using beagles indicated that bilateral PAB using half-closed surgical clips is technically feasible and reproducible, causes less pulmonary arterial intimal proliferation and entails lower risk of residual pulmonary artery stenosis than bilateral PAB using constrictive tapes.

[Strategy for surgery in neonates with coexisting congenital heart diseases].

To determine the most appropriate treatment strategy for neonates with coexisting congenital heart diseases (CHD) requiring surgery, 408 neonates treated in our department between 2002 and 2009 were retrospectively reviewed. Of the 408 neonates, 37 (9.1%) had coexisting CHD. Among the pediatric diseases requiring surgery, esophageal atresia (46.7%), anorectal malformation (24.1%), and tracheobronchial anomalies (75.0%) were frequently associated with CHD, and the survival rate among patients with coexisting CDH was 24.3%, about 2-fold higher than among those without coexisting CHD. Clinical observations in the present series suggested that fatal conditions such as tracheoesophageal fistula should be repaired immediately after birth, followed by palliative cardiac surgery to stabilize the circulatory status if necessary. Thereafter, radical repair of cardiac and pediatric anomalies should be performed, taking into account the balance between surgical stress and cardiac reserve. For this purpose, proper assessment of surgical stress and cardiac function and collaboration among specialists in relevant fields are essential during the perinatal period to determine the treatment strategy for patients with multiple-organ anomalies.

Acute progressive mitral regurgitation resulting from chordal rupture in infants.

This report describes six previously healthy infants with acute mitral valve chordal rupture. Although their initial symptoms were mild respiratory distress and fever, all the infants presented with acute progressive left heart failure and required urgent surgical repair. Mitral valve annuloplasty and mitral valve replacement were performed (for three patients each). Two patients died, both of whom had cardiac arrest and required resuscitation before surgery. Histologic examination of excised mitral valve leaflets showed infiltration of gram-positive bacteria in these two patients. All the patients were 4-6 months old, which may suggest underlying congenital factors. The authors believe early diagnosis and surgery are crucial as a life-saving measure for infants with acute mitral valve chordal rupture.

Cardiac surgery in patients with trisomy 18.

Cardiac surgery is infrequently but increasingly being used to repair congenital heart defects associated with trisomy 18. The clinical details of trisomy 18 patients undergoing cardiac surgery have rarely been reported. Seventeen patients with trisomy 18 and serious cardiac symptoms underwent cardiac surgery in our institution. Age at surgery ranged from 7 to 258 days (median, 66 days). One patient had an atrioventricular septal defect and coarctation of the aorta. The remaining patients had ventricular septal defects, including four patients with coarctation of the aorta. Fourteen patients had associated patent ductus arteriosus. Fourteen patients underwent palliative surgery without cardiopulmonary bypass, and four of these underwent a second-stage intracardiac repair. The other three patients underwent primary intracardiac repair. Postoperatively, 14 patients (82%) were discharged home with improved symptoms. Survival from birth ranged from 12 to 1384 days (median, 324 days). Eight patients survived longer than 1 year. Median postoperative survival was 179 days. Postoperative survival was significantly better after palliative surgery (0 to 1239 days; median, 257 days) than after primary intracardiac repair (1 to 179 days; median, 48 days). Only one patient died of heart failure, suggesting that cardiac surgery was effective in preventing heart failure-related death.