Late complications following surgical repair of total anomalous pulmonary venous return below the diaphragm.

Between May, 1975, and June, 1977, we surgically repaired an infracardiac total anomalous pulmonary venous return in 4 infants under deep hypothermic cardiac arrest. All patients had pulmonary hypertension and a patent ductus arteriosus. All survived operation and required positive end-expiratory pressure with mechanical ventilation. Late complications included patent ductus arteriosus not visualized at initial catheterization, breakdown of an oversewn atrial septal defect repair, and pulmonary venous obstruction despite an anastomosis diamter of 1.5 to 2.5 cm. At reoperation, there was scarring and contraction at the junction of the pulmonary veins and left atrium. One infant with these complications died post-operatively. In another patient, there was kinking of the left lower pulmonary vein at its juncture with the left atrium. In view of these complications we currently recommend the following: ligation of the ductus arteriosus regardless of catheterization findings; a right-sided approach to avoid pulmonary vein kinking; prosthetic patch closure of the atrial septal defect to avoid excessive tension on suture lines and to maintain maximum left atrial size; stellate anastomosis to provide the largest possible drainage channel; awareness that an unusually high positive end-expiratory pressure may be required postoperatively; and early recatheterization.     

Anatomically corrected malposition of the great arteries

We report the case of an infant with anatomically corrected malposition of the great arteries in solitus [S, L, D], associated with ventricular septal defect, patent ductus arteriosus, and right juxtaposition of the atrial appendages. The aortic pole was posterior to the tricuspid mitral line and the conus was bilateral. Successful surgical repair was undertaken by the Senning procedure with closure of ventricular septal defect and division of the ductus. Postoperative cardiac angiography demonstrated no hemodynamic obstruction or residual shunt.     

Unilateral pulmonary vein atresia

A case of unilateral pulmonary vein atresia in a 20-month-old girl is described in this report. A chest X-ray revealed a reticular shadow on the right side, while an electrocardiogram revealed right ventricular hypertrophy. After cardiac catheterization and angiography, a diagnosis of a ventricular septal defect, patent ductus arteriosus, severe pulmonary hypertension, and atresia of the right pulmonary vein was made. The patient underwent an operation for the ventricular septal defect and ligation of the patent ductus arteriosus. During the operation, the right pulmonary vein was found to be atretic, and unrepairable. Pneumonectomy was not performed because the pulmonary arterial pressure decreased to 41 mmHg after the surgical treatment of the intracardiac lesions. Pneumonectomy will be necessary if one or more complications such as persistent hemoptysis, repeated pulmonary infections or the development of pulmonary hypertension, may occur in future.     

Late Complex Biventricular Repair After Bidirectional Cavopulmonary Shunt

Abstract A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow‐up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer® devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows.     

小婴儿巨大室间隔缺损的外科治疗

目的 报告３９例出生６个月以内小婴儿巨大型空间隔缺损的外科治疗经验。方法 体外循环下除４例经肺动脉切口外其余均经右房切口,行补片缝合修补空间隔缺损。其中合并动脉导管未闭５例、房间隔水平分流１２例和右室流出道狭窄２例均同期矫治。结果 ３９例病儿均痊愈出院。结论 巨大型空间隔缺损的小婴儿出现顽固性心力衰竭、药物治疗无明显效果或生长发育停滞及肺动脉高压时即应手术。尽可能完善的围术期处理是降低病死率的重要     

The right-sided window ductus: a rare anatomical entity

A rare type of patent ductus arteriosus called window ductus was identified in association with a right aortic arch in a 1-year-old child who was referred for closure of a membranous ventricular septal defect.     

Reoperation for residual defects following intracardiac repair for tetralogy of Fallot

Reoperation was carried out in two patients who had previously undergone correction for tetralogy of Fallot and presented with severe pulmonary hypertension. One patient who had undergone intracardiac repair five years earlier had a residual ventricular septal defect (VSD), right ventricular outflow (RVOT) obstruction, patent ductus arteriosus and severe pulmonary hypertension. The second patient, who had undergone intracardiac repair four years earlier, also had a residual VSD and RVOT obstruction. Both underwent VSD closure, RVOT resection and pulmonary valve replacement and did well postoperatively. We recommend aggressive surgical treatment in such patients even in the presence of pulmonary hypertension.     

Anomalous origin of innominate artery from right pulmonary artery in DiGeorge syndrome

We experienced a case of anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery). This patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome, who was diagnosed with perimembranous ventricular septal defect, atrial septal defect, and patent ductus arteriosus. This type of anomaly is exceedingly rare.     

External compression of bronchus by aneurysm from divided major aortopulmonary collateral artery after unifocalization.

The right ventricle to pulmonary artery connection with an extracardiac conduit, left pulmonary artery reconstruction, ligation of patent ductus arteriosus, and take-down of right Blalock--Taussig shunt were performed on a 1-year-8-month-old boy who had pulmonary atresia, ventricular septal defect, patent ductus arteriosus, and major aortopulmonary collateral arteries. He previously underwent the unifocalization and right modified Blalock--Taussig shunt at 9 months of age. He repeatedly had a difficulty in weaning from the mechanical ventilator. After removing the aneurysm from the divided major aortopulmonary collateral artery that compressed the left main bronchus externally, it was possible to wean him from the mechanical ventilator.     

Bilateral lung transplant with pulmonary thromboendarterectomy for Eisenmenger's syndrome

Patients with secondary pulmonary hypertension frequently present for evaluation for lung transplantation. In some of these patients, Eisenmenger's syndrome has developed from chronic left to right intracardiac shunts. A smaller group of these patients will also have associated pulmonary artery aneurysms. There is a paucity of literature discussing this topic, however, and currents reports have suggested the need to replace the abnormal pulmonary artery. This paper discusses a patient in whom Eisenmenger's syndrome developed from an atrial septal defect, and resultant pulmonary artery aneurysms and mural thrombi, who underwent successful bilateral lung transplantation with thromboendarterectomy and atrial septal defect closure.     

Interruption of the aortic arch associated with patent ductus arteriosus and ventricular septal defect. Proposal of a new surgical technique for total correction.

A four-year-old girl underwent complete correction of the association of interruption of the aortic arch (Type A, Celoria and Patton's classification), patent ductus arteriosus, and ventricular septal defect. The surgical procedure consists of establishment of a continuity between the ascending and descending aortas utilizing the patent ductus arteriosus and the anterior wall of the pulmonary arterial trunk, reconstruction of the rest of pulmonary arterial trunk with pericardium, and closure of the ventricular septal defect. Though the patient expired from cerebral complications, the hemodynamic result after repair was quite satisfactory. The procedure described makes total correction of this complex anomaly feasible at one operation through a median sternotomy and seems to be a method of choice for most patients with this association of anomalies, unless there is severe narrowing of the patent ductus arteriosus.     

Results with the two-patch technique for repair of complete atrioventricular septal defect

From May, 1982, to September, 1983, 9 patients underwent repair of complete AV septal defect. They ranged in age from 11 months to 48 months and in weight from 5.3 kg to 16.5 kg. Seven patients were 24 months old or less. Previous operations included pulmonary artery banding in 1 patient and ligation of a patent ductus arteriosus with repair of coarctation in another. All patients had large left-to-right shunts (mean pulmonary to systemic flow ratio, 3.1), and the 7 young infants had marked pulmonary hypertension. Mitral regurgitation was absent in 2 patients, mild in 3, moderate in 2, and severe in 2. One patient had the right ventricular dominant form of complete AV septal defect. In all instances, repair was done using separate ventricular and atrial patches. Leaflet tissue was not divided, and a trileaflet mitral valve was left in each patient. Eight patients survived operation and are well 3 to 17 months after repair. The single operative death occurred in the patient with right ventricular dominance. Only 1 patient has mild residual heart failure 4 months after operation. Clinically, mitral regurgitation is absent in 4 patients and, at most, mild in the other 4. No patient has a conduction disturbance. Repair of complete AV septal defect is facilitated by using separate patches for the ventricular and atrial components of the defect. Less distortion is created, and a more accurate reconstruction of a competent trileaflet mitral valve can be done.     

What other anomalies? Failure to wean post ventricular septal defect repair secondary to anomalous origin of the left coronary artery from the pulmonary artery

A six week old infant underwent ventricular septal defect and atrial septal defect closure. Preoperative echocardiography showed evidence of pulmonary hypertension. The post operative course was complicated failure to wean from ventilatory and inotropic support. Echocardiography showed severe left ventricular (LV) dysfunction and suggested some fistulous drainage of the left coronary artery into the right pulmonary artery; this anomalous drainage of the left coronary artery into the right pulmonary artery (ALCAPA) was confirmed with coronary angiogram. Re-implantation of the left coronary artery into the aorta was performed. Extra-corporeal membrane oxygenation (ECMO) was required to allow time for ventricular recovery. Supports were weaned gradually, with concurrent evidence of LV recovery and the child was discharged on postoperative day 30. ALCAPA is rare and typically presents at 8 weeks of age with symptoms of heart failure, as pulmonary pressure falls leading to myocardial ischaemia due to myocardial hypoperfusion with relatively desaturated blood. In our case the pulmonary hypertension and left to right shunt preoperatively were protective, maintaining forward flow of relatively oxygenated blood. While protective to the myocardium this made the preoperative diagnosis of ALCAPA difficult, as there was no flow reversal on Doppler echocardiography. Closure of the septal defects meant this protective effect was lost, with subsequent severe myocardial ischaemia and heart failure. This case highlights the diagnostic challenges of ALCAPA, the 'protective' effects of pulmonary hypertension with ALCAPA, and the importance of early cardiac catheterization in the setting of unexplained failure to wean post cardiac surgery.     

Ductus arteriosus in pulmonary atresia with and without ventricular septal defect. Anatomic and functional differences.

The pulmonary circulation is dependent on the ductus arteriosus in all patients with pulmonary atresia and intact ventricular septum and in some with pulmonary atresia and ventricular septal defect (tetralogy of Fallot type). To assess the time of ductal closure in these two patient categories, we compared the ages at first operation in 58 patients with pulmonary atresia and intact ventricular septum and 32 with pulmonary atresia and septal defect. The age distribution differed significantly between the groups. Whereas 90% of the children with intact ventricular septum required surgery in the first week of life, 50% of those with ventricular septal defect underwent surgery after the first month and 25% after the third month. The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. The phenomenon may have important clinical implications regarding the timing and choice of surgical procedure.     

De Vega tricuspid annuloplasty for tricuspid regurgitation in children

BACKGROUND: Significant tricuspid valve regurgitation (TR) occurs with other congenital heart defects, typically after repair of right-sided obstructive lesions. Since 1991, we applied the De Vega tricuspid annuloplasty technique for TR in children. METHODS: Forty-one children, aged 5 months to 22.7 years (mean, 9.9 years) underwent 42 De Vega tricuspid annuloplasties for moderate or severe TR during correction of other heart defects. One child had a De Vega during primary ventricular septal defect repair. The remaining patients had prior repair of tetralogy of Fallot or pulmonary atresia, or both (19 patients), double-outlet right ventricle (6 patients), pulmonary stenosis (4 patients), pulmonary atresia and intact ventricular septum (3 patients), complete atrioventricular septal defect (3 patients), and other diagnoses (6 patients). At the time of the De Vega, 37 patients (88%) had pulmonary valve replacement or right ventricular to pulmonary artery conduit replacement. Other procedures included aortic or mitral repair or replacement (6 patients), atrial septal defect and ventricular septal defect closure (5 patients), pulmonary arterioplasty (6 patients), and tracheoplasty (1 patient). RESULTS: There were no deaths at follow-up of 3.4 +/- 2.1 years; 1 child required cardiac transplantation 17 months postoperatively. Early postrepair echocardiography quantified TR as absent or mild (34 patients; 81%), mild-to-moderate (4 patients), moderate (3 patients), and severe (1 patient). The most recent echocardiogram showed moderate TR in 11 patients and severe TR in 2 patients (both with recurrent right ventricular hypertension). One child required tricuspid valve replacement 3 years later and 1 child had redo De Vega at the time of conduit re-replacement. No other child has symptomatic TR, significant tricuspid stenosis, or De Vega-related pacemaker implantation. CONCLUSIONS: The De Vega tricuspid annuloplasty safely provides excellent relief of TR, usually in children undergoing pulmonary valve replacement or conduit replacement. Although echocardiographic TR tends to increase with time (especially with right ventricular hypertension), it rarely requires reintervention or causes symptoms.     

A complex case of congenital cardiac anomaly: pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals

Pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals (MAPCAs) is a rare, complex, and heterogeneous congenital cardiac anomaly. The majority of untreated patients present with severe congestive heart failure and respiratory distress in the first decade of life. We describe a 15-year-old cyanotic boy, both of whose pulmonary arteries arise from the arcus aorta via patent ductus arteriosus. In addition to this anomaly, the patient has MAPCAs originating from the descending aorta that perfuse the right upper lobe of the lung, a persistent superior vena cava, an aberrant right subclavian artery, pulmonary atresia, and ventricular septal defect.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

Bilateral lung transplantation with closure of ventricular septal defect in a patient with Eisenmenger syndrome

We report the first case with Eisenmenger syndrome secondary to an isolated perimembranous ventricular septal defect (VSD) in a patient who underwent bilateral lung transplantation and closure of the VSD in Japan. This male patient was diagnosed as having a VSD associated with severe pulmonary hypertension at the age of 7, and right unilateral pulmonary artery banding was performed at age 9. At 38 years of age, we performed bilateral cadaveric lung transplantation with patch closure of the VSD. Explant pathology revealed grade 3 for the right lung and grade 4 for the left lung by the Heath-Edwards classification. The ventricular contractility had gradually improved, and ventricular arrhythmia requiring amiodarone prior to lung transplantation had disappeared. When cardiac function is preserved, bilateral lung transplantation is an option for patients with Eisenmenger syndrome secondary to VSD and should be considered as an alternative to heart-lung transplantation, especially in Japan, where donor organ shortage is critical.     

Neonatal repair of left atrial diverticulum with gigantic thrombus without cardiopulmonary bypass

A 5-day-old neonate with coarctation of the aorta, hypoplastic aortic arch, large apical muscular ventricular septal defect, and patent ductus arteriosus developed pulmonary over-circulation and systemic hypoperfusion underwent bilateral pulmonary artery banding through median sternotomy as a part of hybrid stage I palliation. At operation, left atrial diverticulum with gigantic thrombus formation at the base of the left atrial appendage was incidentally detected by intraoperative direct echocardiography, and therefore, was successfully resected with the whole thrombus inside it without use of cardiopulmonary bypass. Histopathological finding was compatible with diverticulum. The patient was free from atrial arrhythmia and recurrent thrombus formation.     

Complete atrioventricular canal and tetralogy of Fallot: surgical management

Between 1982 and 1989, nine patients with complete atrioventricular (AV) canal and tetralogy of Fallot underwent successful repair of both anomalies. Seven patients had Down's syndrome. One or more previous palliative shunts had been used in six patients. Associated cardiovascular lesions included persistent left superior vena cava (two patients), patent ductus arteriosus with marked stenosis of the left pulmonary artery (one patient). The diagnosis was confirmed by cardiac catheterization and cineangiography. Closure of the ventricular septal defect was performed through a combined right atrial and ventricular approach in each patient. The right ventricular outflow obstruction was relieved with the use of a transannular patch (4), right ventricular infundibular patch (4) or a valved external conduit (1). There were no hospital deaths. One patient died as a result of meningitis 2 years after repair. Progressive left AV valve incompetence developed in one patient requiring valve replacement. A single atrial approach may not provide the best exposure of the subaortic end of the defect. In this regard, we recommend an additional right ventriculotomy in order to ensure safe closure of the septal defect. Although an adequately shaped isolated patch could be sufficient to close the anterior VSD, we favour a separate patch to minimize the possibility of producing subaortic obstruction. A further method to avoid this subaortic stenosis consists of dividing the common anterior leaflet to the right towards the lateral extent of the infundibular septum.