Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center.

BACKGROUND: Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death. METHODS AND RESULTS: We retrospectively reviewed all children diagnosed with left ventricular noncompaction at Texas Children's Hospital from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty-two children were diagnosed with isolated left ventricular noncompaction over the study period. Thirty-one (12.8%) died, and 13 (5.4%) were received a transplant. One hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (hazard ratio, 11; P<0.001). ECG abnormalities were present in 87%, with ventricular hypertrophy and repolarization abnormalities occurring most commonly. Repolarization abnormalities were associated with increased mortality (hazard ratio, 2.1; P=0.02). Eighty children (33.1%) had an arrhythmia, and those with arrhythmias had increased mortality (hazard ratio, 2.8; P=0.002). Forty-two (17.4%) had ventricular tachycardia, with 5 presenting with resuscitated sudden cardiac death. In total, there were 15 cases of sudden cardiac death in the cohort (6.2%). Nearly all patients with sudden death (14 of 15) had abnormal cardiac dimensions or cardiac dysfunction. No patient with normal cardiac dimensions and function without preceding arrhythmias died. CONCLUSIONS: Left ventricular noncompaction has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death.

Continuous-Flow Ventricular Assist Device Support in Adult Congenital Heart Disease: A 15-Year, Multicenter Experience of Temporary and Durable Support.

Heart failure (HF) is common in adult congenital heart disease (ACHD) patients; however, use of continuous-flow ventricular assist devices (CF-VADs) remains rare. We reviewed outcomes of patients with congenital heart disease greater than or equal to 18 years of age at the time of CF-VAD implant at the affiliated pediatric and adult institutions between 2006 and 2020. In total, 18 ACHD patients (15 with great anatomical complexity) received 21 CF-VADs. Six patients (median age 34 years) received seven percutaneous CF-VADs with a median duration of support of 20 days (3-44 days) with all patients survived to hospital discharge and two patients were bridged to durable CF-VADs. Fourteen patients (median age 38 years) received durable CF-VADs. Thirteen patients (93%) survived to hospital discharge and the median duration of support was 25.8 months (6.4-52.1 months). Estimated survival on durable CF-VAD at 1, 3, and 5 years was 84%, 72%, and 36%, respectively. Three patients were successfully bridged to transplantation. Device-related complications include cerebrovascular accident (n = 5), driveline infection (n = 3), device infection requiring chronic antibiotic therapy (n = 4), gastrointestinal bleeding (n = 6), and presumed pump thrombosis (n = 5). These results show percutaneous and durable CF-VADs can support ACHD patients with advanced HF.

Arterial pulse wave analysis: An accurate means of determining cardiac output in children.

OBJECTIVE: Cardiac output is a useful measure of myocardial performance. Standard methods of determining cardiac output are not without risk and can be problematic in children. Arterial pulse wave analysis (PulseCO), a novel, minimally invasive cardiac output determination technique, offers the advantage of continuous monitoring, convenience, and low risk. This technique has not been validated in children. The purpose of this study was to validate PulseCO as an accurate means of noninvasively determining real-time cardiac output in children. DESIGN: Prospective, single-center evaluation. SETTING: Children's hospital. PATIENTS: Any child with a structurally normal heart, undergoing hemodynamic evaluation in the cardiac catheterization laboratory, was included. INTERVENTIONS: A prograde right heart catheterization was performed, and cardiac output was determined using the thermodilution technique, via placement of a pulmonary arterial catheter. MEASUREMENTS AND MAIN RESULTS: Thermodilution results were compared with continuous real-time cardiac output measurements obtained with the PulseCO system, and they were then analyzed by standard correlation techniques and Bland-Altman analysis. Twenty patients were evaluated with a median age of 10.5 yrs and a median weight of 25 kg. The mean thermodilution cardiac index was 3.3 +/- 0.9 L/min/m, whereas the mean PulseCO cardiac index was 3.1 +/- 0.9 L/min/m. Standard Pearson correlation tests revealed a correlation coefficient of .94 (p < .001). Bland-Altman analysis revealed excellent clinical agreement with a mean difference of 0.19 L/min/m and a precision of 0.28 L/min/m at 2 sd. CONCLUSIONS: Arterial pulse wave analysis by the PulseCO system provides a novel, minimally invasive method of determining real-time cardiac output in children.

Late pacemaker requirement after pediatric orthotopic heart transplantation may predict the presence of transplant coronary artery disease.

BACKGROUND: Few data are available regarding pacemaker implantation after pediatric orthotopic heart transplantation. The purpose of this study was to assess the incidence, indications and associations with regard to pacemaker placement in children who have undergone orthotopic heart transplantation. METHODS: We performed a retrospective study of all patients undergoing orthotopic heart transplantation at our institution from October 1984 to March 2001. Data obtained included demographics, indications for pacemaker, presence of transplant coronary artery disease and long-term follow-up. Patients were divided into: Group 1, patients requiring a pacemaker within 3 months of transplantation; and Group 2, patients requiring a pacemaker beyond 3 months. RESULTS: Pacemakers were required in 7 of 106 (6.6%) transplant recipients. Pacing indications for patients in Group 1 (n = 2) were persistent bradycardia with pause-related ventricular arrhythmia and atrial flutter with resultant sinus pauses of up to 4 seconds. In Group 2 patients (n = 5), indications for pacing were high-grade atrioventricular (AV) block in 1 patient and episodic sinus pauses up to 3.3 seconds associated with syncope/dizziness in the remaining 4 patients. All patients in Group 2 had transplant coronary disease diagnosed within 1 year of pacemaker implantation. All had resolution of symptoms and no complications after implantation. CONCLUSIONS: Pacemakers are infrequently required after cardiac transplantation in children. Despite not meeting classic symptomatic sinus bradycardia criteria, pacemaker placement should be considered post-transplantation in patients with episodic sinus pauses and dizziness or syncope. Patients who present with the aforementioned symptoms or high-grade AV block should be evaluated closely for the presence or development of transplant coronary artery disease, as it may be their first symptom.

Staphylococcus aureus Infections in Children With Congenital Heart Disease.

BACKGROUND: Congenital heart disease (CHD) is the most common risk factor for infective endocarditis (IE) in children. Staphylococcus aureus is among the most common organisms to cause IE, yet there are little data describing the risk factors for invasive S aureus disease in children with CHD. We examined the epidemiology of S aureus infections in children with CHD. METHODS: Patients with a history of CHD and S aureus infection were identified from a surveillance study of S aureus infections at Texas Children's Hospital. Clinical and laboratory data from medical records were reviewed. All isolates were screened for the presence of the antiseptic tolerance gene qacA/B. Dichotomous variables were compared with Fisher's exact test and continuous variables with Mann-Whitney U tests, and two-tailed P values of <.05 were considered significant. RESULTS: Two hundred forty-eight S aureus infections developed in 216 patients with CHD. Methicillin resistance was seen in 53.6% of isolates. Surgical site infections accounted for 28.2% of cases and bacteremia accounted for 20.4% of cases. Bacteremia was associated with IE in 29.5% of the episodes. Infective endocarditis was more often associated with prolonged bacteremia, thrombocytopenia, and a higher C-reactive protein (CRP) compared with uncomplicated bacteremia. The qacA/B gene was found in 16.9% of isolates and was associated with bacteremia and prolonged hospitalization. CONCLUSIONS: Staphylococcus aureus is an important cause of morbidity among children with CHD. Infective endocarditis was common with S aureus bacteremia in this population; in addition, prolonged bacteremia, thrombocytopenia, and CRP >10 mg/dL may serve as diagnostic adjuncts for IE. qacA/B-positive isolates are associated with adverse clinical outcomes.