Double Outlet Right Ventricle With Right-Sided Aorta From the Left-Sided Morphologically Right Ventricle in the Setting of Discordant Atrioventricular Connections.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.

Surgical repair for common arterial trunk with pulmonary dominance, hypoplasia of ascending aorta, and interrupted aortic arch.

The arrangement of aortic and pulmonary pathways is extremely variable in the hearts with a common arterial trunk. Almost always, interruption of the aortic arch is seen in the setting of hypoplasia of the ascending aorta and dominance of the pulmonary circulation. This subset poses substantial challenges in surgical repair and portends poor outcomes. In this report, we briefly describe the technique of ascending aorta reconstruction and other aspects of the surgical repair of this rare malformation.

Functionally univentricular heart with systemic venous anomalies: surgical palliation and pulmonary arterial reconstruction with a roll of left atrial appendage.

In this report, we describe a 3-year-old patient with a functionally univentricular heart (UVH), who had a combination of double outlet right ventricle (DORV) along with an unrouteable interventricular communication (VSD), severe infundibular and pulmonary valvar stenosis, and severe left pulmonary artery (LPA) ostial stenosis. This patient also had an interrupted inferior caval vein (IVC) with bilateral superior caval veins (SVC). We were able to undertake a successful Kawashima procedure with interruption of the antegrade pulmonary blood flow, reconstructing the LPA using a pedicled roll of the left atrial appendage (LAA).

Oral thyroxin supplementation in infants undergoing cardiac surgery: A double-blind placebo-controlled randomized clinical trial.

BACKGROUND: Decreases in serum total thyroxin and total triiodothyronine occurs after cardiopulmonary bypass, and is reflected as poor immediate outcome. We studied effects of oral thyroxin supplementation in infants who underwent open-heart surgery. METHODS: In this prospective study, 100 patients were randomized into 2 groups: 50 in the thyroxin group (TH) and 50 in the placebo group (PL). Patients in the TH group received oral thyroxin (5 µg/kg) 12 hours before surgery and once daily for the remainder of their intensive care unit (ICU) stay. Data on intraoperative and postoperative variables were recorded. Cardiac index (CI) was measured. Perioperative serum thyroid hormone levels and serum interleukin-6 and tumor necrosis factor-α were measured. Secondary analysis was performed by dividing patients into simple and complex subcategories. RESULTS: Results of the primary analysis indicated a higher CI in the TH compared with the PL. In the complex category, the mean duration of mechanical ventilation was 3.85 ± 0.93 and 4.66 ± 1.55 days in the TH and PL, respectively (P = .001). Mean ICU stay was 6.79 ± 2.26 and 8.33 ± 3.09 days (P = .03), and mean hospital stay was 15.70 ± 4.77 and 18.90 ± 4.48 days (P = .01) in the TH and PL, respectively. There were no significant differences between the TH and the PL in the simple category. CI was higher in the TH at all time points (P = .004). The average therapeutic intervention scoring system scores for the first 2 days were higher in the PL in the complex category. CONCLUSIONS: Oral thyroxin supplementation improves the CI and reduces the inotropic requirement. In addition, it reduces the duration of mechanical ventilation, ICU and hospital stay, and therapeutic intervention scoring system in infants after surgery for complex congenital heart defects.

Comparison of del Nido and St Thomas Cardioplegia Solutions in Pediatric Patients: A Prospective Randomized Clinical Trial.

We conducted a prospective randomized trial to compare del Nido (DN) cardioplegia with conventional cold blood cardioplegia (St Thomas [STH]) in pediatric patients. We randomized 100 pediatric patients aged ≤12 years undergoing elective repair of ventricular septal defects and tetralogy of Fallot to the DN and the STH groups. In the DN group, a 20 mL/kg single dose was administered. In the STH group, a 30 mL/kg dose was administered, followed by repeated doses at 25- to 30-minute intervals. The primary outcome was cardiac index that was measured 4 times intra- and postoperatively. Troponin-I, interleukin-6, and tissue necrosis factor-alpha were measured. Myocardial biopsy was obtained to assess electron-microscopic ultrastructural changes. Cardiac indices were significantly higher in the DN group than in the STH group 2 hours after termination of cardiopulmonary bypass (P = 0.0006), after 6 hours (P = 0.0006), and after 24 hours (P ≤ 0.0001). On repeated measure regression analysis, the cardiac index was on an average 0.50 L/min/m2 higher in the DN group than in the STH group at any time point (P = 0.002). Duration of mechanical ventilation (P = 0.01), intensive care unit stay (P = 0.01), and hospital stay (P = 0.0007) was significantly lower in the DN group. Patients in the DN group exhibited lower troponin-I release 24 hours following cardiopulmonary bypass (P = 0.021). Electron microscopic studies showed more myofibrillar disarray in the STH group (P = 0.02). Use of long-acting DN cardioplegia solution was associated with better preservation of cardiac index, lesser troponin-I release, and decreased morbidity. Ultrastructural changes showed better preservation of myofibrillar architecture.

Arterial switch operation in patients with transposition and a left-sided aorta.

OBJECTIVES: Arterial switch operation is the treatment of choice in infants with transposed arterial trunks. It is technically challenging to perform in patients having usual atrial arrangement and concordant atrioventricular connections but having a left-sided aorta. Correction in this setting requires surgical expertise and precision. Here we review our experience with such patients. METHODS: Between January, 2002 and October, 2013, the arterial switch operation was performed in 20 patients in the combination emphasised above. Patient records were analysed in detail for coronary arterial patterns, and for the techniques used for transfer of the coronary arteries and reconstruction of the great arteries. Outcomes were recorded in terms of in-hospital survival and left ventricular function at the most recent follow-up. RESULTS: All patients survived the procedure. Ages ranged from 3 days to 18 months, with a median of 75 days; the weight of the patients ranged from 3 to 8.8 kg, with a median of 3.85 kg. The LeCompte manoeuvre was performed in only nine patients. The mean cardiopulmonary bypass time was 157.5±24.9, with a median of 161 minutes, and the mean aortic cross-clamp time was 101.2±23.8, with a median of 102 minutes. Subsequently, two patients died: the first due to a sudden onset of ventricular fibrillation and the second during a crisis of severe pulmonary hypertension. At the last follow-up, which ranged from 23 to 41 months, with a mean of 38.04±2.32 and a median of 38.4 months, all 18 survivors were in NYHA class I, with none requiring cardiac medications and all having normal bi-ventricular function without residual defects. CONCLUSION: With appropriate technical modifications, patients with concordant atrioventricular and discordant ventriculo-arterial connections with a left-sided aorta can undergo successful anatomical repair.

Atrial switch procedure in children more than 5 years of age: mid-term results.

OBJECTIVES: In developing countries, where patients present late, the atrial switch operation is still a preferred palliation for d-transposition of great arteries (d-TGA). In this report, we present our experience in patients with d-TGA who were 5 years of age or older. METHODS: Twenty-seven patients underwent an atrial switch procedure between January 2004 and December 2014. The standard technique consisted of a combination of the Senning and Mustard's repair with Schumacker's in situ modification for construction of the pulmonary venous baffle. RESULTS: The median age was 8 years (mean: 9.42 ± 4.9, range: 5-26 years). Anatomical variations were dextrocardia (n = 3), situs inversus (n = 3), juxtaposed atrial appendages (n = 4) and left superior vena cava (n = 6). Median aortic cross-clamp and bypass times were 63 and 105 min, respectively. The median ventilator support duration was 15 h (mean: 13.7 ± 4.3, range: 6-24 h). The median intensive care unit stay was 2 days (mean: 2.38 ± 0.69, range: 2-4 days). The median hospital stay was 6 days (mean: 6.3 ± 1.7, range: 4-12 days). There were no early or late deaths. The median follow-up duration was 46 months (mean: 55.15 ± 34.71, range: 1-124 months). There were no deaths or re-operations. One patient had mild systemic venous obstruction after 4 years; one underwent embolization of aortopulmonary collaterals after 5 years. The event-free survival rate at 124 months was 90.9 ± 6.13% (95% CI: 68.3-97.65). CONCLUSIONS: The atrial switch operation using the described technique is low risk, carries acceptable results and is a valuable management option in older patients with d-TGA and a regressed LV.

Surgical Challenges of Familial Hypercholesterolemia.

A 21-year-old patient with familial hypercholesterolemia presented with angina caused by ostial stenosis of the left internal mammary artery and severe calcific aortic stenosis with small aortic root 9 years after coronary revascularization. The ostium of the left internal mammary artery was enlarged using a saphenous vein patch through a left supraclavicular incision, which improved left ventricular function. Successful aortic valve replacement with posterior aortic root enlargement was subsequently performed. The surgical management of this condition is discussed briefly.

Atrial Switch Operation in a Late Presenter With d-Transposed Great Arteries, Juxtaposed Atrial Appendages, and Bilateral Superior Caval Veins.

A 26-year-old patient with d-transposition of great arteries (d-TGA), bilateral superior vena cava, and juxtaposed atrial appendages underwent a successful atrial switch operation. It is extremely uncommon to encounter a previously unpalliated patient with d-TGA at this age. Unusual morphologic features in this patient necessitated technical modifications to successfully accomplish an atrial switch procedure.

Anatomical Repair of an Unusual Combination of Tetralogy of Fallot and Atrioventricular Septal Defect With Unroofed Coronary Sinus.

A 30-month-old female was admitted with recurrent spells and severe cyanosis. Preoperative echocardiography was diagnostic of tetralogy of Fallot with an atrial septal defect of the primum type, unroofed coronary sinus, and a left superior vena cava draining into the left atrium. At surgery the patient was found to have a complete atrioventricular septal defect in addition to these anomalies. Complete anatomical correction was achieved through the right atrial approach.

A Simple Technique for Closing Multiple Muscular and Apical Ventricular Septal Defects.

BACKGROUND: Multiple ventricular septal defects (VSDs) are difficult to close. In this report, we describe a simple and safe technique of closing multiple muscular and apical VSDs. METHODS: Between January 2010 and December 2013, 52 patients with a muscular VSD either in isolation or in association with other congenital heart disease underwent surgery using this technique in which a black silk thread was passed through the suspected VSD opening into the left ventricle and brought out through the mitral valve and the interatrial septum. Sutures were placed around the silk thread to close the VSD. RESULTS: Out of 52 patients, 34 were male; age ranged from 45 days to 5.5 years. In addition to the large subaortic/lage muscular VSD, three additional VSDs were present in 34 patients, two additional VSDs were present in 13 patients, and five patients had "Swiss Cheese septum." The VSDs were mid-muscular in 35 patients and were apical in 17 patients. Eleven patients had associated complex lesions. Intraoperative transesophageal echocardiograms did not reveal any significant residual shunt in any of these patients. There was no step-up on oximetry. There was one hospital death due to sepsis. Follow-up was available in 48 patients; three patients were lost to follow-up. At follow-up, no patient had a residual VSD. CONCLUSION: The biventricular approach through trans-right atrial, trans-interatrial septum using a thread through the hole method for closing multiple muscular VSD is effective with no persistent residual defects.