Annals of Pediatric Cardiology最新文献

Truncus arteriosus (TA) is a major cyanotic heart malformation infrequently associated with aortic arch interruption and isolation of the branch pulmonary artery. Anomalous superior vena caval drainage to the left atrium (LA) is hitherto unreported in TA. In a 45-day-old infant diagnosed with TA and cyanosis, anomalous drainage of the right superior vena cava into the LA was not identified preoperatively or intraoperatively. It was detected postoperatively due to persistent hypoxemia. The anomaly posed unique challenges in the subsequent management. This case report highlights the unusual association of systemic venous drainage in TA.

Stridor, choking episodes and recurrent respiratory infections are common causes of referral for echocardiographic evaluation of the cardiovascular system. Vascular anomalies are rare but important causes of airway symptoms and/or feeding difficulty in young infants and should be carefully evaluated during echocardiogram.

We present a case of a 4-year-old boy with posttraumatic non-A non-B aortic dissection. Pediatric aortic dissection is a rare entity with ill-defined risk factors. A high index of suspicion is needed to identify these patients with aggressive management to offset a high mortality rate. Diagnosis was established with a computed tomography scan, which showed a dissection flap in the aortic arch. The child was taken up for surgery and underwent successful aortic arch replacement with arch vessel reimplantation. We report this case not only because of its incidental rarity but also to highlight the meticulous planning and execution that was essential for a successful outcome.

Cardiac rhabdomyomas are the most common primary cardiac tumors observed in neonates and children, which have been frequently associated with the tuberous sclerosis complex. These benign tumors usually have a natural history of regression but can sometimes cause significant clinical issues by obstructing blood flow and arrhythmias. We present an unusual case of a neonate who presented with a large cardiac rhabdomyoma with obstruction to the right ventricular inflow and outflow. The tumor necessitated an urgent surgery, which proved to be technically challenging.

Background: Breastfeeding is recognized as optimal for infants with congenital heart disease (CHD). We sought to evaluate breastfeeding practices among mothers of infants with CHD and identify key modifiable barriers to breastfeeding.

Methods: Initially, a cross-sectional telephone survey was conducted among 100 mothers whose infants had undergone cardiac surgery in the past year. Based on the results of this survey, a structured interview questionnaire was designed and administered to 50 mother-infant dyads who were prospectively recruited from the inpatient population and followed postoperatively.

Results: Fifty infants were included (median age: 4.5 months [interquartile range (IQR): 2-7]; weight: 4.01 kg [IQR: 3.5-5.1]). Before surgery, 40 mothers (80%) were breastfeeding, whereas ten mothers (20%) were not. Notably, 17 mothers (34%) had stopped breastfeeding at some point before surgery, primarily due to perceived inadequate milk supply (82%). Additional reasons for the interruption of breastfeeding included difficulties with infant sucking or latching (35%), the belief that breast milk alone was insufficient (23%), and sore or cracked nipples (17%). Postoperatively, 39 mothers (78%) resumed breastfeeding after 3 days (IQR: 2-4). A postoperative feeding delay, defined as a delay in resuming breastfeeding beyond the anticipated timeframe of 4 days (as determined by the initial telephonic survey), was observed in 8 (16%) cases.

Conclusion: This study identifies the gaps and challenges in breastfeeding practices among mothers of infants with CHD. Targeted interventions are needed to support and sustain breastfeeding practices in these infants, addressing challenges and promoting continued breastfeeding during the postoperative period and beyond.

Most neonates with prenatally detected d-transposition of great arteries with intact ventricular septum (TGA-IVS) have a relatively standard preoperative course, but some patients with restrictive foramen ovale (FO) or ductus arteriosus tend to have severe hypoxemia after birth, posing a significant risk of mortality in the first few hours after birth. Poor mixing of oxygenated and deoxygenated blood leads to severe cyanosis and death. We present two prenatally diagnosed neonates with TGA-IVS who presented with significant desaturation, necessitating emergency balloon atrial septostomy (BAS). In both patients, the resultant mixing was inadequate; ductal stenting had to be resorted to restore oxygenation. These cases highlight the importance of prenatal identification of high-risk features, such as restrictive FO or ductal constriction, in fetuses with TGA-IVS. A small proportion of these babies who have inadequate mixing despite an anatomically adequate BAS can be further stabilized before surgery by means of ductal stenting.

Objective: Ventricular assist device (VAD) implantation is an established treatment for infants with severe heart failure (HF), serving as a bridge to heart transplantation or enabling myocardial recovery. This study aims to evaluate the probability of ventricular recovery following VAD support in infants with severe HF secondary to myocarditis. In addition, we analyze clinical experiences focusing on the weaning process, challenges during explantation, and outcomes, with special attention to a small cohort (n = 4) to highlight specific findings.

Patients and methods: From 1988 to 2024, 63 consecutive pediatric HF patients were supported with VAD systems. This retrospective descriptive study includes four patients (2016-2024) who achieved myocardial recovery and underwent VAD explantation. Weaning assessments included heart catheterization and echocardiography to determine suitability for VAD removal. One patient with congenital heart disease and severe HF was excluded due to prior reporting.

Results: Four patients diagnosed with cardiomyopathy - three with parvovirus B19 confirmed by polymerase chain reaction and one with arrhythmogenic cardiomyopathy - received Berlin Heart^® Excor left VADs. Two children developed frequent valve thrombosis requiring pump replacement. All patients experienced mild-to-moderate neurological complications postimplantation. Renal and hepatic functions normalized by follow-up.

Conclusions: Myocardial recovery during VAD support is possible, particularly in children with severe myocarditis and cardiomyopathy. Concurrent HF medications alongside VAD support appear to promote recovery. Furthermore, VAD therapy improves systemic hemodynamics, contributing to normalization of renal and hepatic functions. These findings support the use of VADs for recovery in selected pediatric patients.

Background: The importance of right-left ventricular interactions is increasingly recognized in patients with congenital heart disease. Interventions to improve right ventricular volume/pressure overloading may result in improvement of left ventricular systolic dysfunction. We hypothesized that patients with right ventricular volume or pressure overloading lesions and left ventricular dysfunction would have improvement in left ventricular function following isolated intervention to improve right ventricular hemodynamics.

Methods and results: Patient records were reviewed to identify participants. Patients with left ventricular dysfunction (left ventricular ejection fraction <55% by echocardiogram) who underwent interventions to improve right ventricular volume or pressure overloading conditions were included. Interventions to improve right ventricular volume/pressure overloading conditions included closure of an atrial septal defect (ASD) and pulmonary valve replacement (PVR). Seventeen patients were identified with right ventricular volume or pressure overloading lesions and left ventricular dysfunction who underwent interventions to improve right ventricular hemodynamics. The majority of patients demonstrated improvement in left ventricular function postprocedure. PVR was performed in 12 patients - 11 of whom were for pulmonary insufficiency and one for pulmonary stenosis (four surgical and eight transcatheter). Five patients had closure of an ASD - two of whom were closed with a transcatheter device and three closed surgically. Seven out of nine transcatheter interventions demonstrated improvement in left ventricular function within 24 h.

Conclusion: Patients with left ventricular dysfunction who undergo interventions to alleviate right ventricular volume or pressure overloading lesions can have rapid improvement in left ventricular function.

The functional integrity of the systemic atrio-ventricular valve (AVV), particularly when tricuspid in morphology, is frequently compromised in patients with a systemic right ventricle (RV), such as those with ccTGA, d-TGA post-Mustard/Senning repair, or Fontan-palliated hypoplastic left heart syndrome. Systemic RV anatomy is associated with adverse long-term outcomes, largely driven by progressive AVV regurgitation and ventricular dysfunction. Unlike the systemic left ventricle (LV), the RV and its associated tricuspid valve have structural and developmental differences that predispose to failure under systemic load. Greater mechanistic understanding is needed to inform prognosis and guide management in this high-risk population.

Introduction: Conventionally, the surgical approach for Taussig-Bing anomaly (TBA) and transposition of the great arteries with aortic arch obstruction (AAO) has often involved a two-stage strategy. However, in contemporary practice, most centers now favor a one-stage repair. Despite this trend, the choice between one-stage and two-stage strategies may still arise in specific anatomical cases, such as those with interrupted AA (IAA) or in low-weight infants.

Patients and methods: We conducted a review of our institutional experience with single-stage correction in 24 consecutive cases of TBA associated with AAO. Each patient underwent a single-stage correction that included an arterial switch operation (ASO) along with ventricular septal defect (VSD) closure through baffling of the left ventricle to the neo-aorta and AA repair. Early mortality or reoperation was defined as death or the need for reoperation occurring prior to hospital discharge or within 30 days following ASO. Reintervention was classified as either a reoperation or a transcatheter procedure.

Results: The study comprised 24 cases. Among them, 6 (25%) patients had TBA with aortic coarctation, 15 (62.5%) patients had TBA with hypoplastic AA (HAA), 2 (8.3%) patients presented with TBA and IAA, and one patient had TBA with HAA alongside partial anomalous pulmonary venous connection. There were two early postoperative deaths (8.3%). Recoarctation occurred in 2 (8.3%) cases, both of which involved TBA with AA hypoplasia. Both patients successfully underwent balloon dilation of the coarctation after 285 and 312 days, respectively. One (4.2%) patient developed subaortic stenosis and underwent a modified Konno procedure after 233 days. The total number of reinterventions was 5 (20.8%), which included three cardiac catheterization procedures (two successful balloon dilations of the coarctation and one unsuccessful balloon dilation of the supravalvular stenosis), along with two surgical reinterventions.

Conclusions: The single-stage repair involving VSD closure, AA reconstruction, and ASO is an applicable and safe option for patients with combined TBA and concomitant AAO. We recorded reinterventions for recurrent coarctation, supravalvular, and subvalvular aortic stenosis. Long-term follow-up is essential, and early percutaneous interventions may help lower the rates of repeat surgeries.