A systematic approach to epicardial echocardiography in pediatric cardiac surgery: An important but underutilized intraoperative tool.

Intraoperative echocardiography is an integral component of the peri-operative management of pediatric heart disease. It confirms the adequacy of surgery, identifies residual lesions, and can provide useful hemodynamic data. It, therefore, helps to decide on the need for revision of repair and guides the postoperative management strategy. Intraoperative echocardiography is done with the use of either an epicardial probe or a transesophageal probe. Epicardial echocardiography is a simple, useful modality and has the ability to perform imaging in cases where transesophageal echocardiography cannot be easily performed, for example, in low birth weight babies. We attempt to describe in detail the technique of epicardial echocardiography and the various views that we have found useful for a complete postsurgical evaluation. The limitations of the technique are also discussed in detail.

Double outlet of both ventricles: morphological, echocardiographic and surgical considerations.

OBJECTIVES: To describe the morphology, echocardiographic features and surgical management of the entity appropriately described as 'double outlet of both ventricles'. METHODS: Seven patients (5 males, age 0.5-7.5 months) were diagnosed to have a unique form of subarterial ventricular septal defect (VSD) and ventriculo-arterial connection, where a muscular outlet septum straddled the crest of the ventricular septum in a cruciate manner, such that both great arteries were equally committed to both ventricles. Diagnosis was established by echocardiography, with 6 patients submitted to surgical repair by means of intracardiac routing of the left ventricle to the aorta using 2 patches. RESULTS: Surgical repair was successful in all 6 patients in whom it was attempted. In addition, 1 patient underwent concomitant repair of aortic coarctation, and 2 had closure of multiple VSDs. We lost 1 patient to follow-up after diagnosis. Follow-up with a range from 3 months to 8 years in the remaining patients revealed all to be clinically well with satisfactory growth of both outflow tracts. CONCLUSIONS: We describe a series of patients with the ventriculo-arterial connection best described as 'double outlet of both ventricles'. Diagnosis is readily established by echocardiography. Good early and midterm results can be expected subsequent to surgical repair using 2 patches for interventricular septation.

Mid-Term Follow-Up of Neonatal Neochordal Reconstruction of Tricuspid Valve for Perinatal Chordal Rupture Causing Severe Tricuspid Valve Regurgitation.

BACKGROUND: Papillary muscle rupture in the perinatal period is a rare event that leads to severe mitral or tricuspid insufficiency due to a flail leaflet. Neonatal tricuspid chordal reconstruction for this condition is rarely reported. Early recognition and treatment have the potential to be lifesaving. We present our surgical experience with five such patients, along with their midterm follow-up. METHODS: Between August 2010 and November 2012, five neonates (aged 1-30 days) underwent surgery for severe atrioventricular valve regurgitation. All neonates had severe tricuspid regurgitation due to ruptured chordae. In addition, two had moderate mitral regurgitation; one due to ruptured chordae of the posterior mitral leaflet and the other due to prolapse of the anterior mitral leaflet. All underwent emergent surgery where the ruptured chordae to the anterior tricuspid leaflet were replaced with neochordae made with expanded polytetrafluoroethylene (ePTFE) suture. The mitral valve was repaired in two patients. RESULTS: All patients survived surgery without the need for postoperative mechanical circulatory assist. Predischarge echocardiograms showed good coaptation of tricuspid and mitral leaflets with minimal regurgitation in all. At follow-up between 75 months to 102 months, four patients had excellent outcomes with less than mild tricuspid regurgitation. One child with flail tricuspid and mitral leaflets developed progressive tricuspid and mitral regurgitation requiring surgical re-repair at 20 months following the initial surgery. CONCLUSION: Repair of chordal rupture of the tricuspid valve in neonates using ePTFE neo-chordae can provide acute salvage and gratifying midterm results in the management of this potentially fatal condition.

Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Through Limited Left Anterolateral Thoracotomy: An Alternative to Repeat Sternotomy.

We report two patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement through a limited left anterolateral thoracotomy. We describe the technique in detail. Both patients were at risk of cardiac injury during repeat sternotomy. This approach reliably avoids the risk of cardiac injury during repeat sternotomy and appears to be safe, simple, and reproducible.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Surgical Options for Uhl's Anomaly.

BACKGROUND: Uhl's anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies. PATIENTS AND METHODS: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty. RESULTS: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up. CONCLUSION: Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.

Off-pump midline repair of coarctation of aorta when associated with intracardiac defects: an alternate approach for single-stage correction.

OBJECTIVES: Strategies for the optimal surgical management of coarctation of aorta with associated intracardiac defects continue to be debated upon. We describe a previously unreported surgical technique for the management of this combination of defects and present our early results. METHODS: Thirty-one patients (median age: 73 days and median weight: 3.9 kg) underwent repair of coarctation of aorta with associated cardiac defects, in a single-stage, through a midline sternotomy. Twenty-eight patients had associated distal arch hypoplasia and 1 had a type I interruption. The coarctation and when necessary, the distal arch, was repaired prior to the institution of cardiopulmonary bypass. Cardiopulmonary bypass was subsequently instituted for the repair of the associated intracardiac defects. RESULTS: All patients had successful repair of coarctation and distal arch hypoplasia without the need for rescue cardiopulmonary bypass. In addition, 29 patients had closure of one or more ventricular septal defects, 3 patients had an arterial switch procedure and 1 had repair of double outlet right ventricle. Repair of supramitral ring and subaortic membrane was performed in another 3 patients. One patient underwent concomitant mitral valve repair. There was one in-hospital mortality (3.22%) due to fulminant sepsis. The median follow-up was 29.5 months (range: 1-58 months). There was no late mortality. Two patients required balloon dilatation for recurrent coarctation. Overall event-free survival rate was 88.5% at both 2 and 3 years of follow-up after surgery. However, intervention-free survival at 2 and 3 years of follow-up was 91.7% among the survivors. CONCLUSION: Repair of coarctation and distal arch hypoplasia or type I arch interruption is feasible prior to the institution of cardiopulmonary bypass without the use of hypothermic circulatory arrest or regional cerebral perfusion, with acceptable and reproducible results.

Large Right Ventricular Clot in Pulmonary Atresia With Intact Ventricular Septum: In Defense of Biventricular Approach.

Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.

Supramitral ring: good prognosis in a subset of patients with congenital mitral stenosis.

BACKGROUND: Management of congenital stenotic mitral valvular abnormalities remains an important therapeutic challenge. Supramitral ring constitutes a small but inadequately described subset that has a relatively good outcome with appropriate management. METHODS: Between 1996 and 2004, 15 patients with supramitral ring were managed in this institution. The demographic and clinical features, diagnostic modalities, morphology of the rings, and the surgical management were studied retrospectively. RESULTS: Accurate preoperative diagnosis was possible by transthoracic echocardiography in 11 patients (73%). The associated anomalies were ventricular septal defects in 8 patients (53%) and abnormalities of the left ventricular outflow tract in 7 patients (47%). A circumferential supramitral ring, separate from the mitral valve, was present in 8 patients (53%). In the remaining, the ring was attached circumferentially to the anterior and the posterior mitral leaflets and was most densely adherent at the posteroinferior commissure in 4 of these 7 patients (57%). Complete excision of ring was possible in all cases, without damage to the mitral valve. There was 1 in-hospital death (6%). At a mean follow-up of 30 months, 14 survivors continue to do well, with no significant recurrence of mitral stenosis. CONCLUSIONS: Patients with supramitral ring constitute a subset of patients with congenital mitral stenosis who have a relatively good prognosis. In many cases, the supramitral ring is entirely separate from the mitral valve, and when attached, it is usually most prominent at the posteroinferior commissure. In both cases, complete resection is surgically feasible and usually provides lasting relief.