Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.

Prenatally diagnosed left atrial appendage aneurysm with various postnatal imaging investigations: A case report.

A congenital left atrial appendage aneurysm (LAAA) is a rare cardiac malformation, that is, usually diagnosed in adulthood. It is rarely diagnosed prenatally. In most cases, surgical resection is recommended soon after the diagnosis has been made due to the risk of arrhythmia and thrombotic events. The present report describes a case of LAAA that was prenatally diagnosed and was asymptomatic postnatally. Imaging revealed the relation of the cardiac and airway structures around the LAAA in detail. The patient underwent surgical resection of the LAAA successfully at 7 months of age and is currently healthy at 5 years of age.

Contained rupture of right ventricular outflow tract after Rastelli-type operation.

This report describes a 3-year-old infant with post-operative mediastinitis complicated by a contained rupture of the right ventricle. A contained rupture is recognised as the huge pulsating prominence of the anterior chest wall. CT confirmed blood communication between the right ventricular outflow tract and the cavity surrounded by the pectoral major musculocutaneous flap. This is a significant case in which severe adhesion between the right ventricle and the musculocutaneous flap could maintain her stable haemodynamics with a pulsating prominence.

A rare case of truncus arteriosus Van Praagh type A3: Prenatal diagnosis and postnatal management.

Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C-E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks' gestation. TrA C-E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks' gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management. .

Successful Truncal Valve Replacement After Truncal Valve Repairs.

The surgical management of severe truncal valvular dysfunction is still challenging in neonates with persistent truncus arteriosus. This report describes a 14-day-old neonate with severe truncal valve insufficiency successfully undergoing truncal valve repairs, and followed by valve replacement at the age of 4 years. The truncal valve was quadricuspid with two large and two small leaflets, and all leaflets had severe dysplastic and myxomatous changes. We performed leaflet extension and bicuspidization valvuloplasty for this valve. This patient obtained somatic growth for 4 years without heart failure symptoms, and safely underwent prosthetic valve replacement. This technique would be effective for truncal valve dysfunction in neonates as the life-saving and the bridging procedure to valve replacement.

Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries.

BACKGROUND: In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch. METHODS: Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock-Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years. RESULTS: There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I. CONCLUSION: Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Cardiac surgical strategy for extremely low-birthweight infants with pulmonary overcirculation.

OBJECTIVES: This study aimed to review the clinical outcomes of staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. METHODS: Six extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation underwent staged cardiac surgery between 2005 and 2017. The median birthweight was 895 g (range 620-990 g), and the median gestational age was 28 weeks (range 23-31 weeks). Clinical outcomes were evaluated, and we focused on pulmonary haemodynamics. RESULTS: Pulmonary artery banding or bilateral pulmonary artery banding was performed as the initial palliation at a median age of 23 days with a median body weight of 880 g. Corrective surgery was performed at a median age of 187 days with a median body weight of 3.9 kg. All of the patients successfully underwent corrective surgery and survived to date. Pulmonary hypertension regressed after corrective surgery in all of the patients, except for 1 patient with severe bronchopulmonary dysplasia. CONCLUSIONS: Acceptable outcomes can be obtained by staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. While early pulmonary artery banding can lead to regression of pulmonary hypertension after corrective surgery, close follow-up is required.

Clinical standard for valve area after common atrioventricular valve plasty for a single ventricle.

OBJECTIVES: To determine a clinical standard for post-repair common atrioventricular valve orifice area based on mid- to long-term valve function in patients with a functional single ventricle. METHODS: The medical records of 19 single-ventricle patients who underwent common atrioventricular valve plasty from July 1988 to January 2013 were retrospectively reviewed. Bivalvation valvuloplasty was performed in 7 patients with relatively hypoplastic leaflets. The relationship between the orifice area of the repaired common atrioventricular valve measured intraoperatively and valve function and ventricular volume in the early postoperative period (median, 9.5 months) and at mid- to long-term follow-up (median, 4 years) were analysed. RESULTS: Post-repair valve area was significantly positively correlated with valve regurgitation severity in the early postoperative period (P = 0.001, r = 0.69) and at mid- to long-term follow-up (P = 0.02, r = 0.57). Patients who did not undergo bivalvation had favourable valve function at mid- to long-term follow-up and in the early postoperative period when the post-repair valve area was 96-136% of the normal mitral valve area. Bivalvation patients had significantly more valve regurgitation in the early postoperative period than patients without bivalvation, despite equivalent repaired valve area (P = 0.02). CONCLUSIONS: The post-repair orifice area of the common atrioventricular valve is significantly related to postoperative valve function. The clinical standard of post-repair valve orifice area might be 96-136% of the normal mitral valve area in patients undergoing repair without bivalvation. Patients undergoing bivalvation require greater reduction to obtain favourable mid- to long-term valve function.

Modified commissural patch repair in a child with active mitral endocarditis.

A 9-year-old patient with massive destruction of the mitral apparatus caused by active infective endocarditis underwent mitral valve plasty using a modified commissural autologous pericardial patch repair. This procedure is a clinically relevant and feasible technique for pediatric patients with active mitral valve endocarditis.

Novel technique for implantation of a cardioverter defibrillator in children.

An 8-year-old boy with hypertrophic nonobstructive cardiomyopathy with ventricular fibrillation underwent implantation of an implantable cardioverter defibrillator. The lead was inserted through a pursestring suture in the right atrial appendage, and the tip of coil was placed in the right ventricular apex under fluoroscopic guidance. Another defibrillation coil was placed in the back of the left atrium and left ventricle by the transverse sinus. The device wrapped in a monofilament mesh sheet was placed in the intraperitoneal space. This case utilized a new technique for an implantable cardioverter defibrillator implantation in a small child.

Efficacy of Damus-Kaye-Stansel procedure in patients with univentricular heart associated with ventriculo-arterial discordance and excessive pulmonary blood flow.

Pulmonary artery banding (PAB) and ventriculo-arterial discordance (VAD) were reported to be risk factors of subaortic stenosis in univentricular heart. The aim of this study was to evaluate efficacy of Damus-Kaye-Stansel (DKS) anastomosis. Of all 89 patients undergoing total cavo-pulmonary connection (TCPC) in our center since April 1996, 38 had VAD with high pulmonary blood flow, and had received PAB. Twenty-one of 38 had undergone DKS anastomosis due to subaortic stenosis or due to morphological hypertrophy of the outlet septum (DKS group); the other 17 had not yet (no-DKS group). Percentage end-systolic volume of the systemic ventricle and percentage subaortic lesion in both groups significantly decreased after TCPC (P<0.01). Pressure gradient across systemic outflow tract after TCPC was low in both groups at rest. The gradient in DKS group did not differ from those in control group with ventriculo-arterial concordance (VAC) (P>0.1). Ventricular outflow tract after DKS anastomosis might behave like that of VAC even when dobutamine is loaded, suggesting that the anastomosis should be carried out in many patients with this entity even if stenosis across systemic ventricular outflow is not significant, considering possible stenosis in the future.

Staged Starnes Operation Preserving Patent Ductus Arteriosus for Neonates with Ebstein's Anomaly and Pulmonary Atresia.

We herein reported 2 successful neonates with Ebstein's anomaly and small pulmonary arteries undergoing Starnes operation preserving the patent ductus arteriosus. Subsequent Blalock-Taussig shunt was carried out 1 or 2 months after the first surgery. One case had already undergone a successful Fontan operation, and the other had a successful bidirectional Glenn shunt so far. This staged Starnes strategy might be a safe and simple choice for neonates with Ebstein's anomaly and small pulmonary arteries.

Evaluation of the portal vein after duodenoduodenostomy for congenital duodenal stenosis associated with the preduodenal superior mesenteric vein, situs inversus, polysplenia, and malrotation.

A male infant weighting 2970 g with total situs inversus, polysplenia, malrotation, duodenal stenosis, and complex cardiac anomalies, was admitted to our hospital. At 4 days of age, he underwent surgery that revealed a blood vessel passing over the duodenum from the mesenterium to the porta hepatis. A loose overbridging duodenoduodenostomy was performed to prevent compression of the vessel. The cardiac anomalies were corrected, and he could eat unrestricted diets. At the age of 1 year and 3 months, a 3-dimensional computed tomographic scan demonstrated that the vessel on the duodenum was the superior mesenteric vein (SMV), and it formed the portal vein with the splenic vein at the porta hepatis. Further, the scan revealed no compression of the SMV at the anastomosis. Doppler ultrasonography revealed a normal portal blood flow of 118.6 mL/min. This report describes the junction between the SMV and the splenic vein in a patient who had the SMV passing over the duodenum from the mesenterium. Correctly, patients previously diagnosed with a preduodenal portal vein could have a preduodenal SMV. The loose overbridging duodenoduodenostomy had advantages not only in passage of the anastomosis but also in maintenance of the portal blood flow for the congenital duodenal obstruction with the preduodenal SMV.

Extracardiac Fontan procedure bridging the vertebra for apico-caval juxtaposition.

For 5 patients with univentricular heart associated with apico-caval juxtaposition, an extracardiac Fontan procedure was carried out using an artificial graft bridging the vertebra to avoid graft compression by the vertebra and the ventricle. For 2 patients representing nonconfluency between the inferior caval vein and the hepatic vein, a hand-made, shoe-tree graft was used. Postoperatively all patients are doing well without a stenotic venous pathway. This extracardiac operation using an artificial graft bridging the vertebra may be advantageous for univentricular heart associated with apico-caval juxtaposition to prevent a postoperative stenotic venous pathway.

Minimal access via lower partial sternotomy for congenital heart defects.

To evaluate the invasiveness of a minimal access approach for simple congenital heart defects, and determine whether it can be regarded as a standard operation, 83 patients with an atrial septal defect and 73 with a ventricular septal defect underwent repair through a minimal skin incision and lower partial median sternotomy. There were no operative deaths, severe intraoperative complications, or conversion to full sternotomy. The clinical course of 106 patients was compared with that of 21 treated using a full sternotomy by the same surgeon; there were no significant differences, except in the operative time for ASD patients. The clinical courses of 2 minimal access subgroups (50 patients operated on by residents and 106 treated by the staff surgeon were compared; operative time, bypass time, ventricular fibrillation time (ASD repair), and cardiac arrest time (VSD repair) were significantly shorter in those operated on by the staff surgeon, but there was no difference in clinical course. The minimal access approach produced good cosmetic results, its invasiveness was similar to that of a full sternotomy, and it may be considered a standard operation for pediatric patients with septal defects.

Bidirectional Glenn shunt with concomitant placement of extra-cardiac graft - preparatory procedure for the future total cavo-pulmonary connection.

We herein present bidirectional Glenn shunt and concomitant placement of extra-cardiac graft with an advantage of preparation for a later extra-cardiac total cavo-pulmonary connection, which was successfully performed for seven children with a univentricular heart.

In situ pericardium repair of pulmonary venous obstruction after repair of total anomalous pulmonary venous connection.

A 13-month-old boy with recurrent pulmonary venous obstruction (PVO) after repair of total anomalous pulmonary venous connection (TAPVC, Darling IIa + Ia) was treated successfully with in situ pericardium repair consisting of unroofing coronary sinus at 2 months. At 8 months, stenosis of the right upper and lower pulmonary veins (PV) and left lower PV were detected, and PVO was relieved via resection of the stenosis site and recutback. Echocardiography 3 months later showed obstructed bilateral PVs and connection between left PVs and vertical veins. At reoperation, we conducted in situ pericardium repair for right PVO and anastomosed left PVs to the left atrial appendage. The postoperative course was satisfactory. Echocardiography 12 months later showed no evidence of PVO, but cardiac catheterization 12 months later showed mild obstruction on the right side and normal venous drainage on the left. Although the long-term prognosis is unknown, this sutureless technique is effective in recurrent PVO.