Pediatric Cardiology

The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ≤2.5 kg throughout staged palliation. We performed a single-center retrospective review. Abstracted data included gestational age, birth weight, presence of noncardiac anomalies, and survival through Fontan. Fifty-two patients met inclusion criteria, with a median birth weight of 2.14 kg and gestational age of 36 weeks. Five patients received comfort care only. Of 47 patients who underwent initial surgical palliation, 51% survived to initial hospital discharge. Birth weight and gestational age (GA) were similar between survivors and nonsurvivors. Compared with survivors, risk factors for death prior to initial hospital discharge were as follows: small for GA (P = 0.005), noncardiac anomalies (P = 0.04), need for post-perative extracorporeal membrane oxygenation (P = 0.0004), and conversion from initial palliation to Sano shunt (n = 5, no survivors). Operative survival following Stage 2 palliation was 91% (21/23) and 94% after Fontan (17/18). Overall survival for palliated patients from birth through Fontan was 36%. Low-birth-weight neonates with HLHS have poor overall survival through the Fontan operation, with highest mortality following Stage 1 palliation. Being small for GA and the presence of noncardiac anomalies are important preoperative risk factors for early mortality.

A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and supplying the lower lobe of the left lung. The venous return into the left atrium was normal. The affected lobe had normal lung parenchyma, and its bronchial tree was connected normally with the left main bronchus. Hence, it was not a sequestrated lobe. The boy underwent surgical lobectomy of the left lower lobe and improved. Anomalous arterial supply of a lobe without sequestration of its bronchial tree is a rare pathologic entity. It also is a very rare cause of congestive heart failure in children. Computed tomographic angiography was a useful tool for evaluation of the intrathoracic anomalous vessel in this case.

This report describes a 1-year exchange between members of two pediatric cardiology centers: one in Canada and one in Australia. Five cardiologists participated in sequence, fully engaging in the activities of the host department. The motivation of the exchange was broadly educational including clinical experience, shared expertise, teaching, and research collaboration. Structured debriefing confirmed the value of the exchange. In addition to the experience of working in a different medical system, eight research papers were developed, with two research projects ongoing as well as subsequent exchanges of nursing and technical personnel. Interchange between two academic departments can add strength to both and allow development of new skills and research activity.

Identification of heart transplant recipients at highest risk for a poor outcome could lead to improved posttransplantation survival. A chart review of primary heart transplantations from 1993 to 2006 was performed. Analysis was performed to evaluate the risk of graft loss for those with a transplantation age less than 1 year, congenital heart disease (CHD), elevated pulmonary vascular resistance (index > 6), positive panel reactive antibody or crossmatch, liver or renal dysfunction, mechanical ventilation, or mechanical circulatory support (MCS). Primary transplantation was performed for 189 patients. Among these patients, 37% had CHD, 23% had mechanical ventilation, and 6% had renal dysfunction. Overall graft survival was 82% at 1 year and 68% at 5 years. The univariate risk factors for graft loss included mechanical ventilation (hazard ratio [HR], 1.9; 95% confidence interval [CI], 1.15–3.18), CHD (HR, 1.68; 95% CI, 1.04–2.70), and renal dysfunction (HR, 3.05; 95% CI, 1.34–6.70). The multivariate predictors of graft loss were CHD (HR, 1.8; 95% CI, 1.02–2.64), mechanical ventilation (HR, 1.9; 95% CI, 1.13–3.10), and the presence of two or more statistically significant univariate risk factors (SRF) (HR, 3.8; 95% CI, 2.00–7.32). Mechanical ventilation, CHD, and the presence of two or more SRFs identify pediatric patients at higher risk for graft loss and should be considered in the management of children with end-stage heart failure.

Cardiac disease has emerged as a leading cause of mortality in Duchenne muscular dystrophy in the current era. This survey sought to identify the diagnostic and therapeutic approach to DMD among pediatric cardiologists in Advanced Cardiac Therapies Improving Outcomes Network. Pediatric cardiology providers within ACTION (a multi-center pediatric heart failure learning network) were surveyed regarding their approaches to cardiac care in DMD. Thirty-one providers from 23 centers responded. Cardiac MRI and Holter monitoring are routinely obtained, but the frequency of use and indications for ordering these tests varied widely. Angiotensin converting enzyme inhibitor and aldosterone antagonist are generally initiated prior to onset of systolic dysfunction, while the indications for initiating beta-blocker therapy vary more widely. Seventeen (55%) providers report their center has placed an implantable cardioverter defibrillator in at least 1 DMD patient, while 11 providers (35%) would not place an ICD for primary prevention in a DMD patient. Twenty-three providers (74%) would consider placement of a ventricular assist device (VAD) as destination therapy (n = 23, 74%) and three providers (10%) would consider a VAD only as bridge to transplant. Five providers (16%) would not consider VAD at their institution. Cardiac diagnostic and therapeutic approaches vary among ACTION centers, with notable variation present regarding the use of advanced therapies (ICD and VAD). The network is currently working to harmonize medical practices and optimize clinical care in an era of rapidly evolving outcomes and cardiac/skeletal muscle therapies.

Experience concerning radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in pediatric patients is limited. In adults, success rates vary widely based on the etiology of the VT. Highest success rates have been achieved in patients without structural heart disease. Between March 1998 and December 1999, five young patients (age, 5 months to 15 years; body weight, 5.5–61.6 kg) underwent RFCA for VT at our institution [structurally normal heart (n = 4), preoperative tetralogy of Fallot (n = 1)]. Monomorphic VT was present in four children, and an infant with MIDAS syndrome had polymorphic VT. Clinical presentation varied: palpitations, n = 2, congestive heart failure, n = 3. All patients had been proven to be unresponsive to one to six (median, three) antiarrhythmic drugs. In all five patients, VT could be successfully eliminated by RFCA after a total of nine (range, 1–4) procedures. Activation mapping and pace mapping were used to identify the anatomical substrate, which was located in the right ventricle/right ventricular outflow tract in all four patients with monomorphic VT and in the left ventricular septum/left ventricular free wall in the infant with polymorphic VT. There were no significant complications in any patient. During follow-up (20–42 months), all patients are in normal sinus rhythm. Left ventricular function recovered in all three patients who had initially presented with congestive heart failure. RFCA can be effective, safe, and life saving in children with medically resistant VT who have not been operated on for congenital heart disease, even when the VT is polymorphic. Although the number of patients is small, RFCA may be the treatment of choice for symptomatic VT in pediatric patients.

Transcatheter patent ductus arteriosus closure (TCPC) is an emerging treatment for low birth weight extremely premature neonates (EPNs). Left pulmonary artery (LPA) and descending aorta (DAO) obstruction are described device-related complications, however, data on mid- and long-term vascular outcomes are lacking. A retrospective analysis of EPNs who underwent successful TCPC at our institution from 03/2013 to 12/2018 was performed. Two-dimensional echocardiography and spectral Doppler velocities from various time points before and after TCPC were used to identify LPA and DAO flow disturbances. A total of 44 EPNs underwent successful TCPC at a median (range) procedural weight of 1150 g (755–2500 g). Thirty-two (73%) patients were closed with the AVP II and 12 (27%) with the Amplatzer Piccolo device. LPA and DAO velocities on average remained within normal limits and improved spontaneously in long-term follow up (26.1 months, range 1–75 months). One patient, who had concerning LPA flow characteristics immediately after device implant (peak velocity 2.6 m/s) developed progressive LPA stenosis requiring stent placement 3 months post-procedure. In the remaining infants, including 7 (16%) who developed LPA and 3 (7%) who developed DAO flow disturbances (range 2–2.4 m/s), all had progressive normalization of flow velocities over time. TCPC can be performed safely in EPNs with a low incidence of LPA and DAO obstruction. In the absence of significant progressive vascular obstruction in the early post-procedure period, mild increases in LPA and DAO flow velocities tend to improve spontaneously and normalize in long-term follow-up.

There have been reports of myocarditis following vaccination against COVID-19. We sought to describe cardiac magnetic resonance (CMR) findings among pediatric patients. Retrospective review at a large academic center of patients clinically diagnosed with post-vaccine myocarditis (PVM) undergoing CMR. Data collected included parametric mapping, ventricular function, and degree of late gadolinium enhancement (LGE). Post-processing strain analysis was performed using feature tracking. Strain values, T1/T2 values, and ventricular function were compared to age- and gender-matched controls with viral myocarditis using a Wilcoxon Signed Rank test. Among 12 patients with presumed PVM, 11 were male and 11 presented after the second vaccination dose, typically within 4 days. All presented with chest pain and elevated troponin. 10 met MRI criteria for acute myocarditis. All had LGE typically seen in the lateral and inferior walls; only five had prolonged T1 values. 10 met criteria for edema based on skeletal muscle to myocardium signal intensity ratio and only 5 had prolonged T2 mapping values. Patients with PVM had greater short-axis global circumferential and radial strain, right ventricle function, and cardiac output when compared to those with viral myocarditis. Patients with PVM have greater short-axis global circumferential and radial strains compared to those with viral myocarditis. LGE was universal in our cohort. Signal intensity ratios between skeletal muscle and myocardium may be more sensitive in identifying edema than T2 mapping. Overall, the impact on myocardial strain by CMR is less significant in PVM compared to more classic viral myocarditis.

The aim of this study was to describe pediatric patients who underwent early postoperative cardiac catheterization after congenital heart surgery, their clinical indications, findings, interventions, and complications in a cardiovascular center. A descriptive retrospective study was performed. All catheterizations performed within 6 weeks after congenital heart surgery between January 2004 and December 2014 were reviewed. We analyzed 101 early postoperative catheterizations. They were performed on median postoperative day five (IQR: 0–39); the median age was 64 days (IQR: 22–240). The most common diagnoses were single ventricle (53%), left heart obstruction (12%), and tetralogy of fallot or pulmonary atresia with ventricular septal defect (11%). Most common indications were persistent cyanosis (53%), low cardiac output (24%), and residual defect on echocardiogram (20%). Most frequent findings during the catheterization were pulmonary artery stenosis (29%), surgical conduit obstruction (12%), and coarctation or hypoplasia of the aorta (11%). Forty-six (45%) procedures involved intervention. Most frequent interventions were pulmonary artery, aorta, and Blalock–Taussig fistula angioplasty with or without stent implantation. There were adverse effects in 11 cases (11%), and 30-day mortality was 28% (28 patients) with the majority unrelated to the catheterization directly. Although early postoperative catheterizations are high-risk procedures, they are currently a very good option to solve acute problems in critically ill patients. This study provides relevant information for a better understanding and approach to this complex group of patients.