Brijesh P. Kottayil

Anomalous origin of left coronary artery from pulmonary artery in older children and adults: direct aortic implantation.

BACKGROUND Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presents rarely in adulthood and is treated by different surgical techniques with varying results. This study was undertaken to evaluate the feasibility of reestablishment of a dual coronary system in older children and adults with emphasis on direct aortic implantation. METHODS From 2002 to 2010, 10 patients aged 10 to 51 years (median age, 24 years) underwent surgical correction of ALCAPA. Median weight was 44.5 kg. All the patients except 2 were symptomatic. Two patients were in atrial fibrillation. Eight patients had varying degrees of mitral regurgitation, which was severe in 2. In all patients, the left coronary artery was arising from the facing sinus of the pulmonary artery. Direct left main coronary implantation into the aorta was feasible in all patients. Mitral valve replacement was done in 4 patients. RESULTS At a median follow-up of 5.5 years there was no new angina or infarction. One patient died 3 months postsurgery due to intracerebral bleed. Echocardiography demonstrated normal antegrade flow in the transferred left coronary artery, with 2 patients having persistent moderate mitral regurgitation. Coronary angiography done on follow-up in one patient revealed a patent transferred left coronary artery with distal development of new atherosclerotic lesion. CONCLUSIONS Direct reimplantation of the ALCAPA is considered technically more difficult and hazardous in adults. With increased experience with coronary transfer technique, direct aortic implantation is feasible in adult cases. This provides a more physiologic correction and reestablishment of a dual coronary system with a better outcome.

Anomalous pulmonary venous connection to superior vena cava: Warden technique.

OBJECTIVE Repair of anomalous pulmonary venous connection (APVC) to superior vena cava (SVC) with internal pericardial patch may be complicated by the obstruction of the SVC or pulmonary veins and sinus node dysfunction. Moreover, the shrinkage of the pericardium may lead to late obstruction. Various techniques are practiced, which have reduced the incidence of obstruction to the venous drainage, but sinus node dysfunction continues to be an issue. We reviewed our experience with the Warden technique, predominantly in children, for various types of APVC to the SVC. METHODS Between 2006 and 2010, 32 patients with anomalous drainage of the pulmonary veins to SVC underwent repair by the Warden technique in our institute. The median age at operation was 4 years (range 3 months-34 years). Partial anomalous pulmonary venous connection (PAPVC) was present in 28 patients, while four patients had total anomalous pulmonary venous connection (TAPVC) to the SVC, where one or more pulmonary veins joined the SVC separately from the entry of the common chamber. Clinical data, echocardiography and operative details were collected from our database, retrospectively. RESULTS At a median follow-up of 24 months, there was no mortality. One patient had transient rhythm disturbance. On follow-up, it was revealed that all patients are in sinus rhythm with no evidence of systemic or pulmonary venous obstruction. CONCLUSION The Warden technique is a simple and effective surgical option, which should be preferred for patients with anomalous drainage of the pulmonary veins to SVC. Though arrhythmias are rare in the early follow-up, longer follow-up is required to rule out their late development.

Tricuspid Endocarditis with Indirect Gerbode: Septal Translocation of Posterior Leaflet

Tricuspid valve endocarditis with acquired Gerbode defect is rare and can be quiet challenging to the surgeon, often requiring complex repair procedures. We present a technique for Gerbode shunt closure using pericardial patch and reconstruction of the tricuspid valve by septal translocation of posterior tricuspid leaflet, which resulted in a good and competent tricuspid valve with no residual shunt.

Ruptured sinus of Valsalva aneurysm: 10-year Indian surgical experience:

Sinus of Valsalva aneurysm is a rare anomaly with a higher incidence in Eastern than Western populations. Recent improvements in diagnostic techniques have resulted in more patients undergoing surgical repair. Uncorrected, the intracardiac shunts and frequently associated cardiac lesions cause a preventable deterioration in heart function. We retrospectively analyzed the data of 33 patients who underwent repair of ruptured sinus of Valsalva aneurysm from May 2000 to January 2010. The aneurysms originated from the right coronary sinus in 24 patients and from the noncoronary sinus in 9, and ruptured into the right ventricle in 21, right atrium in 10, and left ventricle in 2. Operative procedures included simple plication (1), patch repair (32), and aortic valve replacement (10). There were 3 early deaths. The 30 survivors were followed up for 5.4 ± 2.6 years; all had an improvement in functional class, with superior results in those with no aortic regurgitation. Surgical treatment of ruptured sinus of Valsalva aneurysm has an acceptably low operative risk and good long-term symptom-freedom survival. An early aggressive approach is recommended to prevent worsening symptoms and more extensive disease.

Three-dimensional-printed cardiac prototypes aid surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases: Early experience and proof of concept in a resource-limited environment

Introduction: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer.assisted conversion of 3D imaging data into physical “printouts” Healthcare applications are currently in evolution. Objective: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high.resolution medical imaging data. (cardiac magnetic resonance imaging/computed tomography. [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases. (CHDs). Materials and Methods: Five patients with complex CHD with previously unresolved management decisions were chosen. These included two patients with complex double.outlet right ventricle, two patients with criss-cross atrioventricular connections, and one patient with congenitally corrected transposition of great arteries with pulmonary atresia. Cardiac MRI was done for all patients, cardiac CT for one; specific surgical challenges were identified. Volumetric data were used to generate patient-specific 3D models. All cases were reviewed along with their 3D models, and the impact on surgical decision-making and preoperative planning was assessed. Results: Accurate life-sized 3D cardiac prototypes were successfully created for all patients. The models enabled radically improved 3D understanding of anatomy, identification of specific technical challenges, and precise surgical planning. Augmentation of existing clinical and imaging data by 3D prototypes allowed successful execution of complex surgeries for all five patients, in accordance with the preoperative planning. Conclusions: 3D-printed cardiac prototypes can radically assist decision-making, planning, and safe execution of complex congenital heart surgery by improving understanding of 3D anatomy and allowing anticipation of technical challenges.

Surgical repair of double-chambered right ventricle in adulthood

The aim of this study was to investigate a rare subset of adult patients with double-chambered right ventricle, pertaining to clinical presentation, rhythm disturbances, surgical approach, and results of surgical repair. A retrospective review of our records revealed that 14 adults had undergone surgical repair of double-chambered right ventricle from 1990 to 2010. Their median age was 25.5 years, and their median weight was 50 kg. Patients presented with various symptoms, the most common being dyspnea on exertion. The mean systolic pressure gradient across the obstruction was 69.8 ± 23.1 mm Hg. The most common associated anomaly was ventricular septal defect. Significant rhythm disturbances were present in 4 patients. Transatrial repair was undertaken in 5 patients, a combined transatrial and transpulmonary approach was used in 4, and a right ventriculotomy in 5. There was no mortality at a median follow-up of 5.1 years. A mild right ventricular outflow tract gradient was noted in 2 patients. Medical management was required for rhythm disturbances in 2 patients. Double-chambered right ventricle can present in adulthood, often with vague symptoms. The midterm results of surgical correction are satisfactory. Long-term follow-up is necessary to detect late arrhythmias.

Two-ventricle repair for complex congenital heart defects palliated towards single-ventricle repair

OBJECTIVES Complex congenital heart defects that present earlier in life are sometimes channelled towards single-ventricle repair, because of anatomical or logistic challenges involved in two-ventricle correction. Given the long-term functional and survival advantage, we have been consciously exploring the feasibility of a biventricular repair in these patients when they present later for Fontan completion. METHODS Since June 2009, 71 patients were referred for staged completion of the Fontan procedure. Following detailed evaluation that included three-dimensional echocardiography and magnetic resonance imaging, 10 patients (Group 1-median age 6 years) were identified and later underwent complex biventricular repair with takedown of Glenn shunt, while completion of extracardiac Fontan repair was done in 61 patients (Group 2-median age 7 years). RESULTS Two-ventricle repair was accomplished in all the 10 Group 1 patients. One patient developed complete heart block requiring permanent pacemaker insertion. Late patch dehiscence occurred in another (awaiting repair). At a median follow-up of 15 months, there was no mortality among the Group 1 patients and all except for 1 patient were symptom free. There were 2 early deaths (3.3%) in the Group 2 patients. CONCLUSIONS Two-ventricular repair, although surgically challenging, should be considered in all patients with two functional ventricles who come for Fontan completion. Comprehensive preoperative imaging and meticulous planning helps in identifying suitable candidates.

Stage one Norwood procedure in an emerging economy:Initial experience in a single center

Objective: The evolution of surgical skills and advances in pediatric cardiac intensive care has resulted in Norwood procedure being increasingly performed in emerging economies. We reviewed the feasibility and logistics of performing stage one Norwood operation in a limited-resource environment based on a retrospective analysis of patients who underwent this procedure in our institution. Methods: Retrospective review of medical records of seven neonates who underwent Norwood procedure at our institute from October 2010 to August 2012. Results: The median age at surgery was 9 days (range 5-16 days). All cases were done under deep hypothermic cardiopulmonary bypass and selective antegrade cerebral perfusion. The median cardiopulmonary bypass (CPB) time was 240 min (range 193-439 min) and aortic cross-clamp time was 130 min (range 99-159 min). A modified Blalock-Taussig (BT) shunt was used to provide pulmonary blood flow in all cases. There were two deaths, one in the early postoperative period. The median duration of mechanical ventilation was 117 h (range 71-243 h) and the median intensive care unit (ICU) stay was 12 days (range 5-16 days). Median hospital stay was 30.5 days (range 10-36 days). Blood stream sepsis was reported in four patients. Two patients had preoperative sepsis. One patient required laparotomy for intestinal obstruction. Conclusions: Stage one Norwood is feasible in a limited-resource environment if supported by a dedicated postoperative intensive care and protocolized nursing management. Preoperative optimization and prevention of infections are major challenges in addition to preventing early circulatory collapse.

An unusual example of isolated double-orifice tricuspid valve

Double-orifice tricuspid valve (DOTV) is a very rare congenital cardiac anomaly. We report an unusual example of DOTV where one orifice was committed to the left ventricle. This was identified preoperatively and managed surgically.

A life-threatening infective pseudoaneurysm of the left ventricle in a toddler.

Pseudoaneurysms of the ventricle are an uncommon occurrence in children. They may be secondary to previous cardiac surgery or infection of the pericardial space. Infective pseudoaneurysms require urgent surgery because rupture of the pseudoaneurysm may have catastrophic consequences. The outcome with surgery is excellent. We report a toddler with ruptured pseudoaneurysm secondary to purulent pericarditis who recovered with an emergency surgical closure of the opening of the pseudo-aneurysm.