Pediatric Cardiology最新文献

Anomalous origin of coronary arteries from the pulmonary artery (ACAPA) are rare but clinically significant condition with high mortality if left untreated. Even more rarely, ACAPA is associated with other congenital heart defects. From 1974 to 2024, 120 patients with anomalous coronary arteries connected to the pulmonary artery were retrospectively analyzed. Medical records including surgical operative notes and angiography protocols were screened for any other cardiac and extracardiac defects. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) was present in 103 patients, anomalous right coronary artery connected to the pulmonary artery (ARCAPA) in 6, anomalous circumflex coronary artery connected to the pulmonary artery (ACXPA) in 7, anomalous left anterior descending coronary artery connected to the pulmonary artery (ALADPA) in 2, and anomalous single coronary artery connected to the pulmonary artery (ASCAPA) in 2 patients. Anomalous origin of the coronary artery from the pulmonary arteries was associated with other congenital heart defects in 16 patients (13%) and with extracardiac anomalies in 10 patients (8%). Most associated cardiac anomalies were left-sided obstructive defects or shunt-lesions. Patients with ACAPA and associated cardiac defects had poorer perioperative survival. A precise diagnosis of coronary anatomy is crucial for preoperative planning and the success of the surgery of patients with congenital heart defects. In particular, for patients with a challenging postoperative course, an anomalous coronary artery originating from the pulmonary artery should be considered.

Obesity factors into hypertrophic cardiomyopathy (HCM)-related risk as a disease modifying environmental factor. Behaviours such as diet and sleep are seldom reported upon in children with HCM. It was our aim to report on these factors in this population. A multicenter cross-sectional study recruited children and teens with HCM aged 10-19 years old from 10 Canadian pediatric care sites. Patient demographics were obtained from review of medical charts. Participants completed the Healthy Hearts Survey, involving 92 questions related to family health history, personal health, smoking, nutrition, and physical or sedentary activity. A cohort of n = 56 patients with median (IQR) age of 15.5 (13.8-16.8) years were enrolled. Over half (53.6%) were classified as overweight or obese based on BMI, with 75% under activity restriction. Consumption of sugar-rich and starchy foods alongside skipping of meals (43.6%) and snacking (87.5%) were reported. A desire to lose weight was prevalent (48.2%) despite general perceptions of good health. Participants slept a median duration of 8 (7.4-9.0) hours on weekdays, however, sleep disturbances were widely reported. A majority (96.4%) reported less than 20 min of exercise per day over the course of the week. Unhealthy patterns regarding diet, sleep, and physical or sedentary activities that associate with cardiovascular risk are present within children with HCM. These habits may worsen disease burden in children with HCM. It is unclear how activity restriction factors into such patterns. There are modifiable lifestyle factors in children with HCM which, if addressed, could positively impact cardiovascular risk.

Cardiac surgery, both adult and pediatric, has developed very rapidly and impressively over the past 7 decades. Pediatric cardiac surgery, in particular, has revolutionized the management of babies born with congenital heart disease such that now most patients reach adult life and lead comfortable lives. However, these patients are at risk of cerebral lesions, which may be due to perioperative factors, such as side effects of cardiopulmonary bypass and/or anesthesia, and non-perioperative factors such as chromosomal anomalies (common in children with congenital heart disease), the timing of surgery, number of days on the intensive care unit, length of hospitalization and other hospitalizations in the first year of life. The risk of cerebral lesions is particularly relevant to pediatric cardiac surgery given that cerebral metabolism is about 30% higher in neonates, infants and young children compared to adults, which renders their brain more susceptible to ischemic/hypoxic injury. This issue has been a major concern throughout the history of cardiac surgery such that many preventive measures have been implemented over the years. These measures, however, have had only a modest impact and cerebral lesions continue to be a major concern. This is the subject of this review article, which aims to outline these protective measures, offer possible explanations of why these have not resolved the issue, and suggest possible actions that ought to be taken now.

Though several studies have demonstrated that preoperative oral feeding (PO) can be safe in patients with congenital heart disease, they are commonly prohibited from doing so, potentially precluding the development of such skills. We sought to determine whether preoperative oral feeding is associated with freedom from tube feeding at postoperative discharge. Single-center, observational study including patients in the first month of life (≤ 30 days of age) who underwent a single cardiac surgery between 7/1/2017-6/30/2022 and survived to discharge. Preoperative PO was defined as any oral intake up to the day of cardiac surgery. General demographics and peri-operative characteristics were analyzed. A total of 235 patients were included of which 178 (78%) PO fed preoperatively, and 171 (73%) were discharged taking full PO. Those discharged without tube feeds received more preoperative oral feeds, were less likely to have a chromosomal abnormality/genetic syndrome, had lower STAT categories, and were less likely to have postoperative complications, vocal cord dysfunction or prolonged mechanical ventilation. Multivariate analysis found that any preoperative PO [odds ratio (OR) of 2.78 (CI 1.48, 5.24, p-value = 0.002)] and increasing amounts of PO were predictive of full PO intake at discharge [≤ 20 ml/kg/day (OR 2.06, CI 1.03, 4.14, p-value = 0.042) and > 20 ml/kg/day (OR 4.07, CI 1.88, 8.84, p-value = 0.004)]. Preoperative oral feeding is a strong predictor of discharging with full PO intake after cardiac surgery in the first month of life and that it may also improve with increasing volumes. Multi-institutional analyses are warranted.

This proposal presents a proof of concept for the use of pulmonary flow restrictors (PFRs) based on MVP™-devices, drawing from clinical experience, and explores their potential role in the management of newborns with hypoplastic left heart syndrome (HLHS), other complex left heart lesions, and infants with end-stage dilated cardiomyopathy (DCM). At this early stage of age, manually adjusted PFRs can be tailored to patient's size and hemodynamic needs. Although currently used off-label, PFRs have substantial potential to improve outcomes in these vulnerable patient populations. When integrated into a holistic treatment strategy, they represent a promising advancement in care. There is significant potential to reduce mortality and improve the quality of life for neonates with HLHS and variants, as well as to support age-dependent functional regeneration in DCM, all while avoiding the need for advanced surgical procedures, including general anesthesia. The main barrier to broader adoption is the limited availability of appropriately sized MVP devices. However, with ongoing patient-centered knowledge sharing and refinement of transcatheter techniques, there is reason for optimism that PFRs, tailored for individual patients, could provide significant benefits to thousands of infants with congenital heart disease and DCM worldwide.

We sought to evaluate the intracardiac morphology and associated cardiovascular anomalies in patients with double inlet right ventricle (DIRV) on multidetector CT angiography. A retrospective search of our departmental database was conducted from January 2014 to January 2023 to identify patients with a diagnosis of DIRV on CT angiography. The intracardiac anatomy and associated cardiovascular abnormalities were systematically evaluated. Patients with a common atrioventricular valve were excluded. DIRV was diagnosed in 41 patients (36 males, 5 females; mean age at imaging: 7.4 years [range: 4 months-30 years]). Mirror-imaged viscero-atrial arrangement was observed in 12/41 (29.3%) while right isomerism and left isomerism were seen in 6/41 (14.6%) and 2/41 (4.9%) patients respectively. Hypoplastic left ventricular cavity was seen in 39/41 (95.1%) patients. AV valve straddling was observed in 40/41 (97.6%) patients. Atrial septal defect was seen in 28/41 (68.3%) patients. All (100%) patients had presence of ventricular septal defect. Twisted atrioventricular connections were seen in 4/41 (9.8%) patients. The most common ventriculo-arterial morphology was presence of double outlet right ventricle (DORV) seen in 25/41 (61%) patients. Some degree of pulmonary outflow obstruction was seen in 24/41 (58.5%) patients. Systemic venous anomalies, pulmonary venous anomalies and coronary artery anomalies were observed in 13/41 (31.7%), 6/41 (14.6%) and 8/41 (19.5%) patients. DIRV is a type of single ventricle congenital heart disease associated with an array of cardiovascular abnormalities, most commonly DORV and pulmonary outflow tract obstruction. The knowledge of associated anomalies is imperative for preoperative planning. CT angiography allows for detailed anatomical evaluation of DIRV and associated cardiovascular anomalies which may positively impact surgical management in these patients.

Neonates with congenital heart disease (CHD) who undergo cardiopulmonary bypass (CPB) are at high-risk for unfavorable neurodevelopmental (ND) outcomes and are recommended for ND evaluation (NDE); however, poor rates have been reported. We aimed to identify risk factors associated with lack of NDE. This single-center retrospective observational study included neonates < 30 days old who underwent CPB and survived to discharge between 2012 and 2018. Primary outcome (NDE) was ≥ 1 appointment at our center's dedicated cardiac, neonatal, or general ND clinics before the 3rd birthday. Predictor variables included demographic, medical, and social factors. Social disorganization index (SDI) was obtained with geocoding based on address at time of discharge. Logistic regression identified risk factors associated with lack of NDE. The cohort included 594 patients, predominantly male (59%) and white (59%). A majority (63%) had NDE. Lack of NDE was more common in patients with postnatal CHD diagnosis, CHD without arch obstruction, absence of postoperative seizures, living below 100% poverty level, lack of insurance, younger parental age, and overall higher SDI (p < 0.03). In multivariable analysis, lack of NDE was associated with single-ventricle CHD without arch obstruction (OR 2.17; 95% CI 1.08-4.55), two ventricle CHD without arch obstruction (OR 2.56; 95% CI 1.59-4.17), and higher SDI (OR 1.25; 95% CI 1.05-1.49); all p < 0.05. This study identifies medical and neighborhood-level socioeconomic factors that may help address care gaps in this high-risk population. Patients with socioeconomic disparities may benefit from increased care coordination upon discharge.