Extracorporeal membrane oxygenation support for hypertrophic cardiomyopathy in an infant of a diabetic mother.

Infants of diabetic mothers are at risk for transient hypertrophic cardiomyopathy. These infants are typically asymptomatic, but may develop signs of poor cardiac output from left ventricular outflow tract obstruction. This case illustrates the successful use of extracorporeal membrane oxygenation to support cardiac output in an infant of a diabetic mother with outflow tract obstruction until hypertrophy improved.

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

INTRODUCTION: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies. METHODS: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016. RESULTS: Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients' isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, P = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, P < .01), male gender (HR: 3.7, P < .01), and two-ventricle repair (HR: 3.0, P = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years). CONCLUSIONS: This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.

Suprasternal Approach to Transcatheter Aortic Valve Replacement in a Complex Congenital Pediatric Patient Presenting With Cardiogenic Shock.

Transcatheter aortic valve replacement (TAVR) offers well established benefit for adults with severe aortic stenosis, although applications in the pediatric population remain limited. We describe a case of a 15-year-old male with complex congenital heart disease presenting with cardiogenic shock in the setting of mixed severe aortic stenosis (AS) and severe aortic insufficiency (AI). Self-expanding TAVR was performed via suprasternal approach with robust clinical improvement. At one month follow up, he had resolution of clinical heart failure with improvement in ejection fraction and no symptoms of valvulopathy. To our knowledge, there have been no described cases of suprasternal TAVR in a pediatric patient.

Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.

Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms. Her clinical picture was consistent with BD. She was initially treated with prednisone and cyclophosphamide, followed by maintenance therapy with infliximab. This case describes a unique patient with OCA who developed BD with large pulmonary aneurysms and has remained stable for over 1 year while receiving infliximab.

Late Left Ventricular Outflow Tract Obstruction Following the Rastelli Operation: Expectations Out to 20 Years.

BACKGROUND: Consensus is lacking regarding the optimal operation for transposition, ventricular septal defect, and pulmonary stenosis. METHODS: Between 1968 and 2012, a total of 76 patients underwent the Rastelli procedure, with 52 mid- or long-term survivors. A bracketing analysis was used to estimate the likelihood of late left ventricular outflow tract obstruction (LVOTO). RESULTS: Early mortality decreased over the period of study, with no hospital mortality since 2000. Among one year survivors, 10- and 20-year survival was 90% and 72%, respectively. Freedom from reoperation for LVOTO was 87% at 20 years, with no increase in risk among patients having the procedure before 5 years of age. Available late echocardiographic or catheterization data indicated mild or no LVOTO at a median of 14.3 years in a subset of 38 patients. Estimated freedom from major LVOTO at 20 years is bracketed between the estimate of 87% freedom from reoperation for LVOTO at 20 years and the 78% freedom from reoperation for LVOTO or cardiac death by 20 years. CONCLUSION: The Rastelli operation can be performed in the current era with an early mortality that approaches 0% and with 20-year survival that exceeds 70%. The late risk of important LVOTO appears to range from about 13% to 22% at 20 years, with no increase in risk among patients operated upon before the age of 5 years.

Novel technique of valve-sparing aortic root replacement in two children younger than 3 years of age.

A novel technique of valve-sparing aortic root replacement was applied to 2 children younger than 3 years of age with Marfan syndrome and large aortic root aneurysms. Using elements of both the remodeling and reimplantation techniques, circumferential rings from a 20-mm to 22-mm polyester graft provide stabilization at the subannular and sinotubular levels, and bovine pericardial patches create pseudosinuses. Follow-up at 2 years in 1 patient and 7 months in a second patient revealed satisfactory valve function with stable aortic root size.

Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window.

Aortopulmonary (AP) window is an extremely rare congenital anomaly involving a communication between the ascending aorta and pulmonary artery. Two-dimensional transthoracic echocardiography is traditionally used in the diagnosis but has limitations. We report the usefulness of three-dimensional transthoracic echocardiography in the assessment of AP window in two newborns studied by us.

Is the "perfect Fontan" operation routinely achievable in the modern era?

OBJECTIVE: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure. METHODS: Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation. RESULTS: An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a "perfect" outcome. Freedom from replacement or revision of the tube was 97% at 10 years. CONCLUSION: Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the "perfect" outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.

Assessment of surgically unroofed coronary sinus by live/real time three-dimensional transthoracic echocardiography.

Both two-dimensional transthoracic echocardiography (2DTTE) and live/real time three-dimensional transthoracic echocardiography (3DTTE) were attempted in a 25-year-old morbidly obese female with total anomalous pulmonary venous return (TAPVR) into the coronary sinus (CS) in whom surgical unroofing of CS with patch closure of CS ostium was performed in infancy to redirect pulmonary venous flow into the left atrium (LA). The patient had become increasingly symptomatic over the past 1 year because of severe left-to-right shunting due to dehiscence of the patch used to close the CS ostium. Despite a poor acoustic window, 3DTTE was able to identify a communication between the LA and CS which resulted from surgical unroofing of the CS as well as flow signals moving into the right atrium from the CS. These findings were not detected by 2DTTE.

Identification of serious congenital heart disease in neonates after initial hospital discharge.

OBJECTIVE: Neonates with congenital heart disease (CHD) frequently require prompt intervention to improve outcomes and limit morbidities. We sought to determine the time to identification for infants with serious CHD admitted to the State of Alabama's neonatal cardiovascular center and to identify types of cardiac lesions associated with an earlier vs. later diagnosis. DESIGN/SETTING: Medical record review of all infants admitted to Alabama's cardiovascular referral center over a 3-year period. PATIENTS: Neonates with serious CHD admitted for medical/surgical care. RESULTS: In total, 178 neonates were identified with serious CHD. Of these, 146 (82%) were identified postnatally. Thirty-two (21%) of the postnatally detected infants were identified after initial discharge from the hospital. Neonates with aortic arch anomalies presented at a median age of 7 days. CONCLUSION: Identification of infants with serious CHD, especially those with aortic arch anomalies is frequently delayed. These findings support the need for improved methods to detect CHD in the newborn.

Usefulness of live/real time three-dimensional transthoracic echocardiography in the assessment of atrioventricular septal defects.

We studied nine patients (five newborns and infants, two children, and two adults) with atrioventricular septal defects (four complete, one intermediate, and four partial) utilizing live/real time three-dimensional transthoracic echocardiography (3DTTE) and a 4-MHz matrix array transducer. In all patients, 3DTTE provided additional morphological and/or functional information as compared to standard two-dimensional transthoracic echocardiography (2DTTE). 3DTTE may be a useful supplement to 2DTTE in the assessment of atrioventricular septal defects.