Complex Congenital Heart Disease Research Articles

Simple febrile seizure et causa bronchopneumonia with complex congenital heart disease.

Complex congenital heart disease (CHD) in children does not often cause febrile seizures. A child with simple febrile seizures, bronchopneumonia, and complex congenital heart disease is discussed. The report analyzes the causes and proposes preventive measures for febrile seizures, bronchopneumonia, or both. A 2-year-old girl with complex congenital heart disease presented with simple febrile seizures possibly caused by bronchopneumonia. The child was severely malnourished (<-3SD), with a body weight of 7.5kg and a height of 78cm. Diagnostics revealed leukocytosis and neutropenia, with X-ray results showing abnormalities in the lungs and heart (ASD, VSD, and PDA). Treatment included diazepam 5 mg rectally for the first seizure and 1 mg IV for the second seizure, as well as paracetamol infusion 5 ml/4h. Understanding simple febrile seizures triggered by bronchopneumonia in patients with complex congenital heart disease requires an integrated approach for patient management, including comprehensive care. The involvement of medical personnel is an important challenge in preventing recurrence and ensuring optimal patient outcome. Simple febrile seizures are likely caused by bronchopneumonia accompanied by complex congenital heart disease. Her recovery after prompt treatment with diazepam and symptomatic drugs emphasizes the importance of recognizing seizure triggers and managing febrile seizures in children with cardiac anomalies.

Out of the ice age: Preservation of cardiac allografts with a reusable 10 °C cooler

ObjectiveStatic cold storage with ice has been the mainstay of cardiac donor preservation. Early preclinical data suggest that allograft preservation at 10 °C may be beneficial. We tested this hypothesis by using a static 10 °C storage device to preserve and transport cardiac allografts. MethodsIn total, 52 allografts were recovered between July 2023 and March 2024 and transported using a 10 °C storage cooler. Results were compared to a 3:1 propensity match of allografts transported on ice. Patients were excluded for the following reasons: dual viscera transplant, previous heart transplant, complex congenital heart disease, or allograft injury during procurement. ResultsAmong the 10 °C cooler cohort, median total ischemic time was 222 minutes at 10 °C versus 193 minutes on ice (P < .0001). Intraoperative change in lactate was statistically lower at 10 °C (3.6 vs 5.1 mmol/L, P = .0016). Cardiac index score was greater in 10 °C cooler hearts at 24 (3.2 vs 3.0, P = .016) and 72 hours (3.3 vs 2.9, P = .037), despite similar vasoactive inotrope scores. There was no difference in severe primary graft dysfunction (1.9 vs 2.6%, P > .99). 10 °C hearts demonstrated less change in lactate but no difference in vasoactive inotrope scores or cardiac index. In hearts with extended ischemic time, delta lactate was lower in 10 °C cooler hearts. There was no statistical difference in outcomes for donor hearts >40 years old. ConclusionsThis is an early experience of static preservation in a 10 °C cooler. Postoperative allograft function was excellent, and lactate profiles lower in those allografts with extended ischemic times. Static cold storage targeting 10 °C may offer an inexpensive method for extended heart preservation. Further investigation is needed to assess long-term outcomes of 10 °C storage.

Heart rate variability and cognitive functions in adolescents with complex congenital heart disease.

Heart Rate Variability (HRV) originates from the interplay between parasympathetic/sympathetic inputs to the heart, thus serving as an indicator of Autonomic Nervous System regulation. Prior research indicates that decreased HRV, marked by reduced autonomic balance, is related to poorer cognitive performance. While the population with congenital heart disease (CHD) show changes in HRV linked with the heart defect, the association between HRV and cognitive functions in CHD remains unexplored. 46 adolescents with CHD who went through infant open-heart surgery and 64 healthy controls (50.9% males, 12.8 ± 1.4 years) underwent neurodevelopmental testing and photoplethysmograph acquisition. Group differences and associations with cognitive functions were analysed with linear regression. P values were FDR-corrected. Adolescents with CHD showed lower HRV (quantified by high-frequency power) compared to controls (p < 0.001). Lower HRV was correlated with worse executive function (β = 0.24, p = 0.044) and lower IQ (β = 0.26, p = 0.010) in the whole sample and with lower IQ (β = 0.35, p = 0.014) in the CHD group. These associations were robust to confounders, including age, sex, and socioeconomic status. Our findings demonstrate an association between HRV and cognitive functions in adolescents with complex CHD. Early detection of alterations in HRV/autonomic regulation may help to identify children with CHD at risk for cognitive impairments. Adolescents with congenital heart disease (CHD) showed lower heart rate variability (HRV), indicating an imbalanced autonomic nervous system. Lower HRV was associated with lower IQ and executive function (EF) in the whole sample. The association between HRV and IQ was significantly stronger in CHD than in healthy controls. This study provides the first evidence of a link between altered HRV and cognitive impairments in the CHD population. Neurodevelopmental impairments seen in adolescents with CHD could be linked to their altered cardiac autonomic nervous activity, marked by low HRV.

Compromised Cerebral Arterial Perfusion, Altered Brain Tissue Integrity, and Cognitive Impairment in Adolescents with Complex Congenital Heart Disease.

(1) Introduction: Adolescents with complex congenital heart disease (CCHD) show brain tissue injuries in regions associated with cognitive deficits. Alteration in cerebral arterial perfusion (CAP), as measured by arterial transit time (ATT), may lead to perfusion deficits and potential injury. Our study aims to compare ATT values between CCHD patients and controls and assess the associations between ATT values, MD values, and cognitive scores in adolescents with CCHD. (2) Methods: 37 CCHD subjects, 14-18 years of age, who had undergone surgical palliation and 30 healthy controls completed cognitive testing and brain MRI assessments using a 3.0-Tesla scanner. ATT values and regional brain mean diffusivity [MD] were assessed for the whole brain using diffusion tensor imaging. (3) Results: The mean MoCA values [23.1 ± 4.1 vs. 28.1 ± 2.3; p < 0.001] and General Memory Index, with a subscore of WRAML2 [86.8 ± 15.4 vs. 110.3 ± 14.5; p < 0.001], showed significant cognitive deficits in CCHD patients compared to controls. The mean global ATT was significantly higher in CCHD patients versus controls (mean ± SD, s, 1.26 ± 0.11 vs. 1.19 ± 0.11, p = 0.03), respectively. The partial correlations between ATT values, MD values, and cognitive scores (p < 0.005) showed significant associations in areas including the hippocampus, prefrontal cortices, cerebellum, caudate, anterior and mid cingulate, insula, thalamus, and lingual gyrus. (4) Conclusions: Adolescents with CCHD had prolonged ATTs and showed correlation with clinical measurements of cognitive impairment and MRI measurements of brain tissue integrity. This suggests that altered CAP may play a role in brain tissue injury and cognitive impairment after surgical palliation.

Early outcomes of minimal invasive surgery via the axillary approach for complex congenital heart disease at the Cardiovascular Center - E hospital

Objective: This study aims to outline the initial outcomes of implementing minimally invasive surgery via the right axillary approach to completely repair complex congenital heart diseases. Summary: Minimally invasive surgical techniques have gained widespread adoption in managing simple congenital heart diseases such as atrial septal defect, ventricular septal defect, partial atrioventricular septal defect, cor triatriatum, and partial anomalous pulmonary venous return. Postoperative results have demonstrated equivalence, if not superiority, to traditional sternotomy procedures while offering enhanced cosmetic benefits. However, for more intricate congenital heart conditions such as tetralogy of Fallot (TOF), complete atrioventricular septal defect (CAVSD), and Ebstein syndrome, this study evaluates the early outcomes of employing minimally invasive surgery via the right axillary approach, a technique that remains relatively uncommon globally. Our study seeks to assess the initial outcomes of this approach in managing complex congenital heart diseases. Subjects and methods: descriptive method based on a series of cases. From April 2023 to December 2023, 45 patients diagnosed with complex congenital heart disease underwent minimally invasive surgery utilizing the axillary approach. This cohort comprised 40 patients with tetralogy of Fallot, 3 patients with complete atrioventricular septal defect, and 2 patients with Ebstein syndrome. Results: All procedures were technically successful, without requiring sternotomy. The cardiopulmonary bypass times for the TOF, complete AVSD, and Ebstein syndrome groups were 81 minutes, 91 minutes, 96 minutes, respectively. Corresponding aortic cross - clamp times were 70 minutes, 72 minutes, and 74 minutes. There were no postoperative fatalities or significant complications. Postoperative echocardiographic evaluations demonstrated favorable outcomes. Conclusion: Minimally invasive surgery via the axillary approach for managing complex congenital heart diseases yields promising early results and offers notable cosmetic advantages.

Effects of an Online Health Management Program for South Korean Adolescents with Complex Congenital Heart Disease during Their Transition to Adulthood: A Randomized Controlled Trial.

Adolescents with complex congenital heart disease (CHD) are at risk of experiencing complications later in life. The purpose of this study was to develop an online health management program for adolescents with complex CHD and to evaluate its effects on self-efficacy, health behavior, and health-related quality of life. A randomized controlled trial design was used. A total of 29 adolescents with complex CHD were divided into an experimental group of 15 and a control group of 14. Participants in the intervention group took part in the 4-week online health management program (weekly online group sessions, 1:1 phone coaching, dietary diary feedback, and provision of health information) developed based on self-efficacy theory, while those in the control group received standard medical follow-up. Data were collected from August 2021 to March 2022 using a questionnaire-including the Korean Self-Rated Abilities for Health Practices: Health Self-Efficacy Measure (K-SRAHP) and Pediatric Cardiac Quality of Life Inventory (PCQLI)-and an ActiGraph accelerometer to track physical activity and sleep. The intervention group showed significant improvements in health self-efficacy (p = 0.003), psychosocial impact (p = 0.013), daily step counts (p = 0.011), and moderate to vigorous-intensity physical activity (p = 0.027). Additionally, a decrease in weekend leisure time sedentary behavior (p = 0.035) was observed. However, there were no significant differences in sleep behavior between two groups. The online health management program significantly enhanced self-efficacy, health behavior, and psychosocial impact in adolescents with complex CHD. These findings will inform the development of policies for transitional medical care tailored to adolescents with complex CHD.

Experience of percutaneous closure of small to large patent ductus arteriosus with Nit-Occlud® device in a tertiary referral hospital in Colombia.

The decision to close patent ductus arteriosus should always be individualized and taken together with the child's family once the risks and benefits of both choices have been exposed. This study aims to report the experience and outcomes in patients undergoing endovascular closure of small to medium-size PDA with a Nit-Occlud® device in a tertiary referral hospital in Colombia. Longitudinal descriptive study, which included all patients under 18 years of age who underwent percutaneous ductal closure with Nit-Occlud® device between January 1, 2011, and February 1, 2023. Patients with associated complex congenital heart disease requiring surgical management, pregnant patients, and patients with incomplete data regarding studied variables were excluded from the study. Eighty-seven patients were documented, with a mean age, weight, and height at closure of 51 months, 14 kg, and 95.83 cm, respectively. About 70% of the patients (n = 61) were female, 76% were under 6-years-old and only one patient was over 15. The average size of the ductus at the pulmonary end was 2 mm. Four of the total number of patients did not achieve PDA closure during the procedure. Of the remaining 83, complete immediate closure was achieved in 81 patients. A device exchange for a larger device was required during the same procedure in one of the cases. Two patients presented residual shunt of 0.5 mm during follow-up, and one required a new procedure for device closure 10 months later. Only one device presented repeatedly embolization to the aorta, requiring surgical removal. As a technical difficulty, one device presented repeated passage into the aorta, so it was decided to remove it before releasing it to avoid complications, and given the complex anatomy of the ductus, surgical closure was indicated. Among the complications, one patient presented a hematoma of the subcutaneous tissue in the right thigh, which improved with medical management, and no deaths related to the procedure were registered. Using the Nit-Occlud® device to close small to moderate-sized ductus remains a safe and effective strategy with successful closure rates at 1-year follow-up irrespective of age, weight, height, or whether it involves a small or medium-sized duct. Despite our limitations, results concerning adverse effects are comparable to those observed in multicentric studies conducted in other regions.

Enhancing discharge preparation for parents after complex cardiac surgery: evaluation of an e-learning resource for nurses.

Parents need to be appropriately prepared by knowledgeable healthcare professionals before going home with their infant following cardiac surgery for complex congenital heart disease (CHD). A quality improvement project was undertaken between 2018 and 2021 to equip healthcare professionals including children's cardiac nurses with the knowledge required to use the Congenital Heart Assessment Tool (CHAT) to teach parents how to monitor their infant at home. The project involved developing, implementing and evaluating an e-learning resource that included simulated scenarios captured on video. An online survey showed that users perceived the e-learning resource as having a positive effect on their understanding of complex CHD and their practice of preparing parents for discharge and home monitoring.

Shone syndrome: a complex and rare congenital heart disease

Shone syndrome: a complex and rare congenital heart disease

The Role of Cardiac MRI and Late Gadolinium Enhancement Grading Protocol in Evaluation of the Right Ventricle in Patients with Repaired Fallot Tetralogy

Abstract Background Tetralogy of Fallot represents 7–10% of congenital heart diseases occurring in 0.5/1000 live births and is the second most common form of complex congenital heart diseases. The four elements of Tetralogy of Fallot: (a) sub-pulmonary obstruction resulting in (b) right ventricle hypertrophy, and (c) malalignment ventricular septal defect resulting in (d) apparent overriding of the aorta over both ventricles. Primary cardiac repair for Fallot Tetralogy patients includes right ventricular out flow tract widening interventions and ventricular septal defect closure. For such patients right ventricular assessment in the postoperative period is essential in aiding further management planning. The purpose of this study is to emphasize the role of cardiac MRI in functional and structural assessment of the right ventricle in Fallot tetralogy patients after surgical management. Results The current study included 30 Fallot Tetralogy patients underwent cardiac MRI after surgical management. The majority of patients 26 out of 30 (86.6%) showed right ventricular dilatation. Four patients had right atrial dilatation. Less than one-third of patients 8 out of 30 (26.6%) had right ventricular systolic dysfunction. The majority of patients 29 out of 30 (96.6%) had main pulmonary artery regurgitation. More than half of the patients 18 out of 30 (60%) had main pulmonary artery stenosis. The majority of patients 25 out of 30 (83.3%) had right ventricular outflow tract late gadolinium enhancement and 18 patients (60%) had base of interventricular septal enhancement. Conclusions MRI is an extremely useful imaging method for functional and structural assessment of the right ventricle after surgical repair of Fallot tetralogy patients with absence of ionizing radiation.

Phenotypic clustering of repaired Tetralogy of Fallot using unsupervised machine learning

ObjectiveRepaired Tetralogy of Fallot (rTOF), a complex congenital heart disease, exhibits substantial clinical heterogeneity. Accurate prediction of disease progression and tailored patient management remain elusive. We aimed to categorize rTOF patients into distinct phenotypes based on clinical variables and variables obtained from cardiac magnetic resonance (CMR) imaging. MethodsA retrospective observational cohort study of rTOF patients with at least two CMR assessments between 2005 and 2022 was performed. From patient records, clinical variables, CMR measurements, and electrocardiogram data were collected and processed. Baseline and follow-up variables between subsequent CMR studies were used to assess both inter- and intrapatient disease heterogeneity. Subsequently, unsupervised machine learning was performed, involving dimensionality reduction using principal component analysis and K-means clustering to identify different phenotypic clusters. ResultsIn total, 155 patients (54.2 % male, median 14.9 years) were included and followed for a median duration of 9.9 years. A total of 459 CMR studies were included in analysis for the identification of phenotypic clusters. Following analysis, we identified four distinct rTOF phenotypes: (1) stable/slow deteriorating, (2) deteriorating, structural remodeling, (3) deteriorated indicated for pulmonary valve replacement, and lastly (4) younger patients with coexisting anomalies. These phenotypes exhibited differential clinical profiles (p < 0.01), cardiac remodeling patterns (p < 0.01), and intervention rates (p < 0.01). ConclusionsUnsupervised machine learning analysis unveiled four discrete phenotypes within the rTOF population, elucidating the substantial disease heterogeneity on both a population- and patient-level. Our study underscores the potential of unsupervised machine learning as a valuable tool for characterizing complex congenital heart disease and potentially tailoring interventions.

Application of right ventricular to pulmonary valved conduit in the surgical treatment of congenital heart disease.

Pulmonary valve replacement and right ventricular outflow tract reconstruction with valved conduits have been the shortcomings of paediatric cardiac surgeons in the treatment of CHD. In recent decades, encouraging achievements have been made in right ventricular outflow tract technology. Since Klinner reported the first right ventricle-to-pulmonary artery connection using unvalved conduits made of autologous pericardium in 1964, various right ventricle-to-pulmonary artery conduits have gradually been used in the treatment of various complex CHD. Compared with other materials, valved homograft conduit (VHC) is more consistent with physiological characteristics, better haemodynamics, easy suture and good haemostasis, anti-calcification, anti-infection, and without the need for lifelong anticoagulation, which makes VHC the best material for reconstruction of right ventricular outflow tract. However, due to the shortage of donor sources, other alternative conduits such as polytetrafuoroethylene valved conduits have been developed, and the results are not inferior to VHC in clinical application. The emerging tissue engineering technology is expected to utilise recipient-derived endothelial cells for implantation onto the decellularized VHC or degradable synthetic materials in order to construct a recipient-specific tissue-engineered valved conduit. This advancement holds great potential as an ideal biological transplant material and valve replacement for CHD. It will completely solve the problems of immune rejection and the growth of the conduit that cannot adapt to the physical growth of children. This review provides a comprehensive review of the clinical indications for right ventricle-to-pulmonary artery conduits application, optimal timing for surgery, current practices in utilising various types of external conduits, and considerations for re-replacement.

Surgical Outcomes of Congenital Heart Disease in the Eastern Region of Libya

Congenital heart disease (CHD) refers to a group of structural abnormalities in the heart that are present at birth. However, due to advancements in medical and surgical care, more children with CHD in developed countries are now surviving into adulthood. This study aims to assess the outcomes of surgically repaired congenital heart disease in the eastern part of Libya. Data was obtained from the medical records of 374 patients, of which 54.2% were male and 45.7% were female, who underwent surgical correction and palliative cardiac surgery between February 2015 and February 2019. The majority of patients were from Benghazi and Al-Jabal Al-Akhdar, and the most common diagnoses were ventricular septal defects (VSD) (25.4%) and complex congenital heart disease (CCHD) (21.9%). The death rate in atrioventricular canal (AVC) defects was 7.4%, CCHD had a mortality rate of 4.8%, and the total mortality rate was 2.9%. In the eastern part of Libya, pediatric cardiac surgery and cardiac surgical intensive care for children with congenital heart disease (CHD) still heavily rely on foreign cardiac surgery missions. There is a need for a comprehensive treatment plan and the development of local capabilities to manage CHD patients independently.

BeGraft Aortic Stents: A European Multi-Centre Experience Reporting Acute Safety and Efficacy Outcomes for the Treatment of Vessel Stenosis in Congenital Heart Diseases.

Stent implantation has become the preferred method of treatment for treating vessel stenosis in congenital heart diseases. The availability of covered stents may decrease complications and have an important role in the management of patients with complex anatomy. This study aims to evaluate the feasibility and safety of the pre-mounted cobalt-chromium stent-graft-covered ePTFE Aortic BeGraft in a broad spectrum of vascular lesions. This is a multicenter retrospective results analysis of 107 implanted BeGraft stents between 2016 and 2022 in six different European centers. One hundred and four patients with a mean age of thirteen years (range 1-70 years) and with the body weight of 56.5 kg (range 11-115 kg) underwent the BeGraft stent implantation. Stents were implanted in the following conditions: aortic coarctation (74 patients), RVOT dysfunction (12 patients), Fontan circulation (7 patients), and miscellaneous (11 subjects with complex CHD). All the stents were implanted successfully. The median stent diameter was 16 mm (range 7-24 mm), and the median length was 39 mm (range 19-49 mm). Major complications occurred in five subjects (4.7%). During a median follow-up of fourteen (1-70) months, stents' re-dilatation was performed in five patients. The BeGraft stent can be used safely and effectively in a wide spectrum of congenital heart diseases. Whether these good results will be stable in the longer term still needs to be investigated in a follow-up given its recent introduction into clinical practice, in particular regarding stent fracture or neointimal proliferation.

Anesthesia in Emergency PDA Stenting with Pulmonary Arteri Atresia

Pulmonary atresia with an intact ventricular septum is a condition that is characterized by a complete obstruction to right ventricular outflow. This condition is fatal if untreated. Ductus arteriosus communicate between pulmonary artery and proximal descending aorta. It is essential in fetal life as a communicating shunt to send blood from right ventricle bypassing the underdeveloped lungs. Ductal patency may be lifesaving in few cyanotic congenital heart diseases to maintain pulmonary blood flow.Case Illustration : We present a case of a 1‑day‑old, 3405 g child who presented to casualty with bluish discoloration. The child had central cyanosis, tachypnea with saturation 80% on 1 L/minute nasal cannula and desaturate to 40%. Transthoracic echocardiography (TTE) revealed intact intraventricular septum with pulmonary atresia and patent ductus arteriosus (PDA). The patient scheduled to an emergency PDA stenting. A heating blanket is installed on the operation table, oxygen saturation with Pulse‑oxymeter, noninvasive blood pressure, end-tidal CO2 monitor, and temperature monitoring were done. Anesthesia induction was performed with 50% oxygen in air, fentanyl 3 µg/kg, ketamine 2 mg/kg, atracurium 0.5 mg/kg and maintained with sevoflurane. The procedure was performed about 20 to 30 minutes. After procedure, oxygen saturation was increased up to 60%. The child was successfully extubated after 24 hours in the Neonatal Intensive Care Unit (NICU) with saturation 92%.Conclusion: PDA stenting is a less invasive palliative procedure for ductal dependent complex congenital heart diseases. Anesthesiologist's role were maintaining arterial saturation and hemodynamics, securing vascular assess, maintaining temperature. It needs thorough understanding of underlying physiology.

The role of natural compensatory mechanisms in complex congenital heart disease: haemodynamic evolution and clinical outcomes in a case of Cor triatriatum sinistrum syndrome.

The role of natural compensatory mechanisms in complex congenital heart disease: haemodynamic evolution and clinical outcomes in a case of Cor triatriatum sinistrum syndrome.

Standardization in paediatric echocardiographic reporting and critical interpretation of measurements, functional parameters, and prediction scores: a clinical consensus statement of the European Association of Cardiovascular Imaging of the European Society of Cardiology and the Association for European Paediatric and Congenital Cardiology.

This document has been developed to provide a guide for basic and advanced reporting in paediatric echocardiography. Furthermore, it aims to help clinicians in the interpretation of echocardiographic measurements and functional data for estimating the severity of disease in different paediatric age groups. The following topics will be reviewed and discussed in the present document: (i) the general principle in constructing a paediatric echocardiographic report, (ii) the basic elements to be included, and (iii) the potential and limitation of currently employed tools used for disease severity quantification during paediatric reporting. A guide for the interpretation of Z-scores will be provided. Use and interpretation of parameters employed for quantification of ventricular systolic function will be discussed. Difficulties in the adoption of adult parameters for the study of diastolic function and valve defects at different ages and pressure and loading conditions will be outlined, with pitfalls for the assessment listed. A guide for careful use of prediction scores for complex congenital heart disease will be provided. Examples of basic and advanced (disease-specific) formats for reporting in paediatric echocardiography will be provided. This document should serve as a comprehensive guide to (i) structure a comprehensive paediatric echocardiographic report; (ii) identify the basic morphological details, measures, and functional parameters to be included during echocardiographic reporting; and (iii) correctly interpret measurements and functional data for estimating disease severity.

Delivering care to women with congenital heart disease: the role of clinical nurse specialist

Congenital heart disease is now the most common reason for women to attend a high-risk joint cardiac–obstetric clinic. With advances in medical care and surgical techniques, most children born with congenital heart disease reach childbearing age and have a good prognosis for long-term survival with a good quality of life. Pregnancy remains a major life event for these women, posing additional risks with the potential for severe morbidity and mortality. The hemodynamic changes induced by pregnancy can unmask undiagnosed congenital heart disease or exacerbate pre-existing conditions. Therefore, specialist care is essential for a successful pregnancy outcome. Women with complex congenital heart disease embarking on pregnancy need the support of a multidisciplinary team, including cardiologists, obstetricians, anaesthetists, midwives and adult congenital heart disease clinical nurse specialists. Clinical nurse specialists in particular play a crucial role in coordinating, supporting, educating and advocating for the mother and fetus through preconception, pregnancy, the puerperium and beyond.

Defining the Typical Course of Persistent Pulmonary Hypertension of the Newborn: When to Think Beyond Reversible Causes

ObjectivesTo describe the typical clinical course of reversible PPHN from perinatal etiologies and compare that with the clinical course of PPHN due to underlying fetal developmental etiologies. Study designThis was a single-center, retrospective cohort study of liveborn newborns either born or transferred to our facility for higher level of care between 2015 through 2020 with gestational age ≥35 weeks and a clinical diagnosis of PPHN in the electronic health record (EHR). Newborns with complex congenital heart disease and congenital diaphragmatic hernia were excluded. Using all data available at time of collection, newborns were stratified into 2 groups by PPHN etiology – perinatal and fetal developmental causes. Primary outcomes were age at initiation, discontinuation, and total duration of extracorporeal life support (ECLS), mechanical ventilation, supplemental oxygen, iNO, inotropic support, and PGE. Our secondary outcome was age at echocardiographic resolution of pulmonary hypertension. Groups were compared by t-test. Time-to-event Kaplan Meier curves described and compared (log-rank test) discontinuation of each therapy. ResultsSixty-four (72%) newborns had perinatal etiologies whereas 24 (28%) had fetal developmental etiologies. The resolution of perinatal PPHN was more rapid compared with fetal developmental PPHN. By 10 days of age, more neonates were off inotropes (98% vs 29%, p<0.01), decannulated from ECLS (100% vs 0%, p<0.01), extubated (75% vs 37%, p<0.01), and had echocardiographic resolution of PH (35% vs 7%, p=0.02). ConclusionsAn atypical PPHN course, characterized by persistent targeted therapies in the second week of life, warrants further work-up for fetal developmental causes.

Transcatheter Embolization in Congenital Cardiovascular Malformations—Variable Use of Vascular Plugs

Objective: The objective of this study is to evaluate the clinical application and primary outcome of transcatheter embolization using Amplatzer™ Vascular Plug (AVP) Type 2 and Type 4 in different congenital cardiovascular malformations.Design: This is a single-center retrospective observational cohort study.Methods: We analyzed clinical and imaging data of 36 patients retrospectively who received transcatheter embolizations of the following malformations using AVP: systemic-to-pulmonary collateral arteries (SPCA), patent ductus arteriosus (PDA), ventricular septal defects (VSD), and aberrant pulmonary sequestration arteries (PSA). We included all patients treated in our institution from January 2010 to July 2023.Results: In 36 patients (median age 40.0 months, range 0.5 months–42.0 years; 56.8% male), 44 AVPs were implanted in 37 procedures. The target lesions were SPCA in n=15 procedures, PDA in n=9, VSD in n=9, and PSA in n=4. Thirty-four AVP Type 2 and 10 AVP Type 4 were applied, the latter only in SPCA and PSA. SPCA was most common in complex congenital heart disease with univentricular physiology (75.0%). VSD were associated with additional cardiac malformations in 33.3%, PDA were associated with prematurity (55.6%), and all pulmonary sequestrations occurred in scimitar syndrome. Primary total or subtotal occlusion succeeded in n38/44 (86.3%). For residual PDA, an additional occluder was implanted in one patient. In one case, pulmonary sequestration had to be treated surgically. One premature infant with PDA closure sustained a relevant obstruction of the left pulmonary artery by the outer AVP disc which required surgical correction 4 months later.Conclusion: Embolization using AVP is a suitable approach for closure of various cardiovascular malformations with a high primary success rate and low complication rate. It should be considered in treatment of different irregular vessel anomalies and in selected VSD.