Cardiol Young [JOURNAL]
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200 papers
Variability in intraoperative and postoperative fluid removal strategies in neonates undergoing cardiac surgery and associations with clinical outcomes.
Stegmeier N, Carlisle A, Alten J … +8 more , Borasino S, Chakraborty A, Gist K, Selewski D, Reichle GW, Zang H, Zender J, Bertrandt R
Cardiol Young · 2026 May · PMID 42136570 · Publisher ↗
BACKGROUND: Various fluid removal strategies are utilized to mitigate the deleterious effects of fluid accumulation in neonates following cardiac surgery. We aim to describe practice patterns and identify associations of...
BACKGROUND: Various fluid removal strategies are utilized to mitigate the deleterious effects of fluid accumulation in neonates following cardiac surgery. We aim to describe practice patterns and identify associations of fluid management strategies with outcomes in neonates undergoing cardiac surgery using the Neonatal and Pediatric Heart and Renal Outcomes Network dataset. METHODS: Multicentre retrospective study including neonates who underwent cardiac surgery between 2015 and 2018. Primary outcomes were duration of mechanical ventilation, delay in first negative daily fluid balance, peak cumulative fluid balance, and severe persistent acute kidney injury. Multivariable logistic and ordinal regression models assessed associations between fluid removal strategies and clinical outcomes. RESULTS: Of 2,240 neonates, 25% ( = 560) received furosemide in the operating room. Peritoneal catheters were placed in 24% ( = 527), and 8.6% ( = 193) underwent prophylactic peritoneal dialysis. Diuretic use on postoperative day 0 ranged 6-95% across institutions. Diuretics on postoperative day 0 were associated with shorter time to negative daily fluid balance (odds ratio 0.55; 95% confidence interval 0.30-0.995) and shorter duration of mechanical ventilation (odds ratio 0.58; 95% confidence interval 0.39-0.86). Intraoperative diuretics were also associated with shorter duration of mechanical ventilation (odds ratio 0.60; 95% confidence interval 0.42-0.86). Receipt of continuous diuretic infusion on postoperative day 0 or 1 was associated with longer duration of mechanical ventilation (odds ratio 3.02; 95% confidence interval 1.98-4.58). No association of diuretic use with severe persistent acute kidney injury was found. CONCLUSIONS: Early use of diuretics in neonatal cardiac surgery is highly variable and has a differential association with outcomes. Future studies that standardize the dose, timing, and mode of administration (intermittent vs continuous) of diuretics are warranted to identify potential associations with outcomes.
Early-onset pheochromocytoma with a somatic mutation in an 8-year-old boy with unrepaired pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
Egashira H, Hosaka S, Nozaki Y
Cardiol Young · 2026 May · PMID 42136564 · Publisher ↗
We report the case of an 8-year-old boy with unrepaired cyanotic CHD who developed pheochromocytoma and presented with hypertensive heart failure. Imaging revealed a tumour in the right adrenal gland. A genetic analysis...
We report the case of an 8-year-old boy with unrepaired cyanotic CHD who developed pheochromocytoma and presented with hypertensive heart failure. Imaging revealed a tumour in the right adrenal gland. A genetic analysis identified a somatic mutation. This case highlights the potential for early pheochromocytoma development in patients with unrepaired CHD and severe cyanosis. This case provides a hypothesis-generating observation suggesting that chronic hypoxia may act synergistically with a pseudohypoxia mutation, potentially leading to an earlier-than-expected presentation of pheochromocytoma.
Optimal dosing of enoxaparin in children with CHD less than 1 year of age.
Price KR, Zaki H, Hussain H … +5 more , Sterner-Allison J, Giannopoulos Figg H, Jahan A, Woods G, Branstetter JW
Cardiol Young · 2026 May · PMID 42136562 · Publisher ↗
INTRODUCTION: Venous thromboembolism prevalence amongst paediatric patients with CHD has increased over the years. Enoxaparin's favourable pharmacokinetic and pharmacodynamic properties, paired with less intensive monito...
INTRODUCTION: Venous thromboembolism prevalence amongst paediatric patients with CHD has increased over the years. Enoxaparin's favourable pharmacokinetic and pharmacodynamic properties, paired with less intensive monitoring parameters, make it a desirable treatment option. Currently, reported enoxaparin dosing strategies and their correlation to therapeutic anti-Xa levels are variable for infants aged 2 to 12 months. METHODS: This retrospective chart review evaluated the percentage of patients who achieved initial target anti-Xa levels on therapeutic enoxaparin. Patients were divided into standard-dose enoxaparin of 1 mg/kg every 12 hours and high-dose enoxaparin of 1.5 mg/kg every 12 hours. RESULTS: Eighty-five patients were included in this study with similar demographics. More patients in the high-dose group achieved initial therapeutic levels of enoxaparin (36 (69%) vs 5 (15%); < 0.001). The time between initial dose of enoxaparin and first therapeutic anti-Xa level was longer in the standard-dose group (87 hrs (IQR 41 to 112) vs 24 hrs (IQR 16 to 40; < 0.001)), and there was no difference in the incidence of minor bleeding (6 (18%) vs 4 (7.7%); = 0.18) or major bleeding (1 (3%) vs 0 (0%); = 0.39), respectively. CONCLUSIONS: High-dose enoxaparin in infants with CHD resulted in a higher percentage of initial anti-Xa target attainment and a decreased time to target anti-Xa level, with no difference in bleeding. Our study suggests it may be safe and effective to dose enoxaparin higher in infants with CHD; however, further studies should confirm these findings.
Successful surgical management of a rare left ventricular pseudoaneurysm following balloon aortic valvuloplasty in an infant.
Beyter MB, Demiroglu MO, Ozdemir R … +3 more , Bulut MO, Akyüz M, Işık O
Cardiol Young · 2026 May · PMID 42130344 · Publisher ↗
Left ventricular pseudoaneurysm is an exceedingly rare, life-threatening complication following percutaneous balloon aortic valvuloplasty. We report a 1-month-old infant presenting with a large left ventricular pseudoane...
Left ventricular pseudoaneurysm is an exceedingly rare, life-threatening complication following percutaneous balloon aortic valvuloplasty. We report a 1-month-old infant presenting with a large left ventricular pseudoaneurysm after successful valvuloplasty for critical aortic stenosis. Multimodal imaging confirmed the diagnosis. The patient underwent successful surgical resection and bovine pericardial patch repair. This case emphasises the importance of early surgical intervention for favourable outcomes.
Does preservation of antegrade pulmonary blood flow reduce pulmonary arteriovenous malformations after bidirectional Glenn? A retrospective observational study.
Talwar S, Kumar TS, Bhende VV … +1 more , Krishnakumar M
Cardiol Young · 2026 May · PMID 42116531 · Publisher ↗
BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are a recognised cause of progressive cyanosis following bidirectional superior cavopulmonary anastomosis (BDG). The role of antegrade pulmonary blood flow (APBF)...
BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are a recognised cause of progressive cyanosis following bidirectional superior cavopulmonary anastomosis (BDG). The role of antegrade pulmonary blood flow (APBF) in preventing PAVM formation remains controversial. While preserved APBF may improve oxygen saturation and pulmonary artery growth, its protective effect against PAVMs is uncertain. Increasing evidence suggests that exclusion of hepatic venous blood from the pulmonary circulation, rather than absence of pulsatile flow, may be the primary mechanism underlying PAVM development. Therefore, management of APBF after BDG should be individualized. METHODS: This retrospective observational study included children with univentricular physiology who underwent BDG before 4 years of age with a minimum follow-up of two years. Patients were divided into two groups based on the presence or interruption of APBF. Clinical data, oxygen saturation, echocardiographic findings, pulmonary artery dimensions, and imaging evidence of PAVMs were analysed using computed tomography angiography and/or cardiac catheterisation. Outcomes were compared between groups. RESULTS: Sixty patients were included. PAVMs occurred in 26.7% of patients with interrupted APBF and 23.3% with preserved APBF ( = 0.766). Oxygen saturation was higher in the preserved APBF group but not statistically significant. Pulmonary artery dimensions and pressures were comparable, and all patients remained suitable for Fontan completion. Cyanosis was more frequent in the APBF-interrupted group. CONCLUSIONS: Preserved APBF improves oxygen saturation but does not reduce PAVM incidence. Hepatic venous exclusion appears to be the primary determinant of PAVM development, supporting individualized surgical decision-making.
Transjugular closure of a large patent ductus arteriosus in a 1100-g infant.
Kanaan M, Ostermayer S, Kosmac B … +2 more , Lawrenz W, Herberg U
Cardiol Young · 2026 May · PMID 42112613 · Publisher ↗
The patent ductus arteriosus is the most common cardiac anomaly in neonates. Haemodynamically significant patent ductus arteriosus is associated with increased morbidity and mortality in preterm babies. Transfemoral veno...
The patent ductus arteriosus is the most common cardiac anomaly in neonates. Haemodynamically significant patent ductus arteriosus is associated with increased morbidity and mortality in preterm babies. Transfemoral venous access is the standard route for patent ductus arteriosus device closure, even in very low birth weight infants. The transjugular approach is also an alternative, but it is more challenging, especially in preterm infants. We report a successful transjugular closure of a patent ductus arteriosus in a 1100‑g infant (the smallest worldwide), where transfemoral access was not possible due to bilateral iliofemoral venous thrombosis.
Gap between guidelines and practice: echocardiography reporting in neonatal pulmonary hypertension.
Gremont S, Ronkainen E, Kallio M
Cardiol Young · 2026 May · PMID 42112612 · Publisher ↗
Targeted neonatal echocardiography is increasingly integrated into neonatal care and plays a crucial role in the evaluation and management of persistent pulmonary hypertension of the newborn. The objective of this study...
Targeted neonatal echocardiography is increasingly integrated into neonatal care and plays a crucial role in the evaluation and management of persistent pulmonary hypertension of the newborn. The objective of this study was to assess the utilisation of echocardiography and quality of reporting the findings in a tertiary neonatal ICU, with reference to recent imaging guidelines. All neonates ( = 49) who required inhaled nitric oxide for persistent pulmonary hypertension at Oulu University Hospital from September 2016 to September 2021 were included in this retrospective study. Altogether, 113 echocardiography evaluations were performed during their treatment. Patient characteristics, treatment outcomes, and details of echocardiography reports were systematically collected and evaluated. Transthoracic echocardiography was performed prior to the start of nitric oxide treatment in all except two critically ill neonates. Echocardiography evaluations were mostly performed by neonatologists (42%), and indications for imaging were diagnostic initial evaluation and treatment follow-up in 49% and 51% of occasions, respectively. The most commonly reported parameters were the patency of arterial duct (88%) and the pattern of ductal shunting (75%), while right ventricular function was reported in only 16% of the reports. Conclusion: Echocardiography was widely used by all specialists involved in the treatment of neonatal pulmonary hypertension but there was a large variation in quality of reports. Allocating resources for structured training and implementing simple, priority-based guidelines, supported by documentation templates and brief, systematic assessment guidance for common clinical scenarios, might improve the quality of reporting.
Thoracic endovascular aortic repair for high-risk Stanford type B aortic dissection in a 15-year-old patient with genetic mutations: a case report with follow-up of four years.
Qiu XX, Hu YP, Li DL
Cardiol Young · 2026 May · PMID 42098984 · Publisher ↗
This case report presents mid- to long-term outcomes of thoracic endovascular aortic repair for high-risk type B aortic dissection in a 15-year-old patient with myosin heavy chain protein 11 genetic mutations. A CT scan...
This case report presents mid- to long-term outcomes of thoracic endovascular aortic repair for high-risk type B aortic dissection in a 15-year-old patient with myosin heavy chain protein 11 genetic mutations. A CT scan showed the primary entry tear located on the lesser curvature near the left subclavian artery, and the dissection originated from the descending aorta to the abdominal aorta, accompanied by 80% narrowing of the true lumen. Following successful endovascular therapy using stent-grafts, the patient recovered well. A four-year follow-up CT scan showed significant thoracic aorta remodelling.
Six-hour renal resistive index as an early marker of acute kidney injury after infant cardiac surgery: a prospective cohort study.
Elsaoudi AR, Kazzaz Y, Ismail SR … +7 more , Jijeh AMZ, Shaath GA, Altaweel H, Hijazi O, Qadi AMH, Kabbani MS, Hamadah HK
Cardiol Young · 2026 May · PMID 42093208 · Publisher ↗
BACKGROUND: Acute kidney injury after infant congenital cardiac surgery is common and worsens outcomes. Renal resistive index may aid early postoperative risk stratification. OBJECTIVE: To evaluate a single six-hour post...
BACKGROUND: Acute kidney injury after infant congenital cardiac surgery is common and worsens outcomes. Renal resistive index may aid early postoperative risk stratification. OBJECTIVE: To evaluate a single six-hour postoperative renal resistive index as an early acute kidney injury marker and its association with postoperative haemodynamic support. METHODS: A prospective single-centre cohort of neonates and infants < 12 months ( = 41) was classified as acute kidney injury ( = 19) or non-acute kidney injury ( = 22). Renal resistive index was measured at six hours (primary), 48 hr, and discharge. Postoperative acute kidney injury was diagnosed using Kidney Disease: Improving Global Outcomes serum creatinine and urine output criteria over 48 hr. Vasoactive-inotropic score was recorded, and discrimination was assessed using receiver operating characteristic analysis. RESULTS: At six hours, renal resistive index was higher in acute kidney injury vs. non-acute kidney injury (0.84 ± 0.07 vs. 0.74 ± 0.08; = 0.0002) with good unadjusted discrimination (area under the curve 0.83; 95% CI 0.68-0.97). Surgical complexity was higher in the acute kidney injury group (median STAT 4 vs. 3). At 48 hr, renal resistive index remained higher ( = 0.017; area under the curve 0.70), while discharge values were similar ( = 0.18; area under the curve 0.62). A six-hour renal resistive index < 0.80 excluded moderate-to-severe acute kidney injury, whereas ≥ 0.86 identified a higher risk. Vasoactive-inotropic score at 48 hr was higher in acute kidney injury (7.2 ± 8.1 vs. 1.9 ± 3.4; = 0.009), and six-hour renal resistive index correlated modestly with inotropic burden ( = 0.56; < 0.001). Three acute kidney injury infants required peritoneal dialysis, all with six-hour renal resistive index ≥ 0.90. CONCLUSIONS: A six-hour renal resistive index may help early acute kidney injury risk assessment after infant cardiac surgery. External validation is warranted.
Systematic evaluation of extracardiac findings on cardiac CT angiography in children with CHD: a large single-centre experience.
Genc HZ, Guzelbag AN, Kangel D … +5 more , Karimov E, Yucel ED, Hatemi AC, Ozturk E, Bas S
Cardiol Young · 2026 May · PMID 42093207 · Publisher ↗
INTRODUCTION: Cardiac CT angiography has seen a steady increase in use for the evaluation of CHD in children. While its primary role is to assess cardiovascular structures, CT angiography also captures extracardiac regio...
INTRODUCTION: Cardiac CT angiography has seen a steady increase in use for the evaluation of CHD in children. While its primary role is to assess cardiovascular structures, CT angiography also captures extracardiac regions, often revealing previously unsuspected abnormalities. This study sought to determine the prevalence, classification, and clinical relevance of extracardiac abnormalities detected in paediatric patients undergoing CT angiography for CHD evaluation. MATERIALS AND METHODS: A retrospective review was conducted on CT angiography examinations from 1336 patients, aged from newborn to 17 years, at a single tertiary care institution. Extracardiac findings within the thoracic and abdominal regions were systematically identified, categorised, and analysed statistically. RESULTS: Extracardiac abnormalities were common, predominantly affecting the respiratory system, including pneumonia (14.5%), atelectasis (13.3%), and pulmonary oedema (11.5%). Airway changes included bronchial malposition (23.3%) and peribronchial thickening (8.3%). Abdominal anomalies were also detected, the most common being hepatomegaly (13.1%), splenomegaly (6.9%), and horseshoe kidney (5.7%). Several of these findings were clinically important and had the potential to affect patient management. DISCUSSION: Extracardiac anomalies are a common finding in paediatric CT angiography performed for CHD evaluation and can have significant clinical consequences. Radiologists and clinicians should follow a systematic approach that evaluates both cardiovascular and extracardiac structures to improve diagnostic accuracy and optimise patient care. In addition to common thoracic and abdominal abnormalities, CT angiography enabled the identification of rare and complex extracardiac anatomical patterns, underscoring its value as a comprehensive imaging modality in paediatric CHD.
Double trouble: double aortic arch with bilateral interruption and bilateral arterial ducts.
Salhadar K, Langley SM, McKenna D … +2 more , Anderson RH, Prasad D
Cardiol Young · 2026 May · PMID 42093206 · Publisher ↗
Double aortic arch with bilateral interruptions is a rare abnormality. Indeed, to the best of our knowledge, it has not previously been reported before. Rare variants, such as a double arch with ligamentous atresia of th...
Double aortic arch with bilateral interruptions is a rare abnormality. Indeed, to the best of our knowledge, it has not previously been reported before. Rare variants, such as a double arch with ligamentous atresia of the left component and double arch with bilateral coarctation, have received previous attention. These rare examples can pose significant diagnostic challenges for those relying on transthoracic echocardiography. Use of cardiac CT or MRI is now critical for prompt surgical planning and correction.
Larger lung volumes in fetuses with transposition of the great arteries and an intact ventricular septum compared to heart healthy fetuses and fetuses with transposition of the great arteries and a ventricular septal defect.
Krogh E, Hellmuth SG, Jørgensen DS … +5 more , Kelly B, Vejlstrup N, Petersen O, Hjortdal VE, Lauridsen MH
Cardiol Young · 2026 May · PMID 42093204 · Publisher ↗
BACKGROUND: Complex CHDs may impair organ development. One proposed mechanism is an altered relationship between blood flow, oxygen delivery, and subsequent organ growth. In this study, we examined whether fetal lung, in...
BACKGROUND: Complex CHDs may impair organ development. One proposed mechanism is an altered relationship between blood flow, oxygen delivery, and subsequent organ growth. In this study, we examined whether fetal lung, intracranial, liver, and kidney volumes differ among fetuses with transposition of the great arteries with an intact ventricular septum, transposition of the great arteries with a ventricular septal defect, and healthy controls. METHODS: Eleven fetuses with transposition of the great arteries (6 with a ventricular septal defect and 5 with an intact ventricular septum) and 22 healthy controls were scanned between 1 and 3 times at gestational age 27-38 weeks, using fetal MRI. We measured lung, total intracranial, liver, and kidney volumes and compared fetuses with and without transposition of the great arteries while subsequently correcting for ventricular septal defect/intact ventricular septum status, estimated fetal weight, and gestational age, using mixed effects regression analysis. RESULTS: Fetuses with transposition of the great arteries+intact ventricular septum had significantly larger lung volumes compared to controls. After adjusting for estimated fetal weight and gestational age, median lung volume ratio (transposition of the great arteries+intact ventricular septum vs. controls) was 1.30 (95% CI: 1.08-1.57; = 0.005). No difference was found in lung volume between fetuses with transposition of the great arteries+ventricular septal defect and controls. No significant differences in total intracranial, liver, and kidney volumes were found between transposition of the great arteries+ventricular septal defect, transposition of the great arteries+intact ventricular septum, and controls. CONCLUSION: In this preliminary study, late-gestation fetuses with transposition of the great arteries-intact ventricular septum had a 30% larger lung volume compared with both transposition of the great arteries-ventricular septal defect and healthy controls. Together with existing evidence of higher fetal pulmonary blood flow and increased oxygen saturation in transposition of the great arteries-intact ventricular septum, these findings support a potential link between blood flow, oxygen delivery, and organ growth.
Navigating downtime as a cardiac data abstractor: ensuring data quality and registry submission.
Schukei S, Shores K, Morris M … +5 more , Khanal D, Well A, Affolter JT, McCullum E, Mavroudis CD
Cardiol Young · 2026 May · PMID 42089173 · Publisher ↗
INTRODUCTION: Electronic medical records (EMRs) have become foundational to healthcare, improving communication, data access, and patient outcomes. However, increasing reliance on EMR's has increased vulnerabitlity durin...
INTRODUCTION: Electronic medical records (EMRs) have become foundational to healthcare, improving communication, data access, and patient outcomes. However, increasing reliance on EMR's has increased vulnerabitlity during downtime. In paediatric cardiac care, where patients require highly specialized, multidisciplinary treatment, the absence of a functional EMR significantly disrupts documentation workflows and threatens the accuracy of data submitted to national cardiac registries including the Paediatric Cardiac Critical Care Consortium and the Society of Thoracic Surgeons. METHODS: This article examines the impact of an unexpected 27-day complete EMR downtime followed by a 10-day partial downtime, where a data abstraction team manually managed data for 123 unique cardiac patient encounters totalling 762 patient days at a paediatric heart centre and the response of the cardiac data abstraction team. We describe how the team adapted its abstraction process during the downtime, used collaborative strategies, enhanced paper tracking, and proactively communicated to maintain data integrity. Efforts were grounded in a deep understanding of paediatric registry metrics and submission requirements. RESULTS: Despite significant workflow disruptions, the team was able to preserve data accuracy and meet registry deadlines by identifying documentation gaps, supplementing data from paper records, and coordinating with frontline providers. The event revealed key vulnerabilities in downtime preparedness but also demonstrated the value of dedicated data abstractors in ensuring continuity of quality reporting. CONCLUSION: Downtime events highlight the critical role of data abstractors and the need for institutional planning and registry-level guidance. Developing robust downtime protocols and embedding abstraction-aware workflows can mitigate documentation risks and protect data quality, ultimately supporting improved outcomes for paediatric cardiac patients.
Postnatal manifestation of left heart failure following foetal-onset right heart failure in biventricular noncompaction with an variant: a case report.
Yamada Y, Hirono K, Yasuda K
Cardiol Young · 2026 May · PMID 42089169 · Publisher ↗
We report a case of foetal-onset biventricular noncompaction with an variant. Prenatal fetal hydrops and right heart failure resolved after birth, but progressive left heart failure developed at 5 months. This transitio...
We report a case of foetal-onset biventricular noncompaction with an variant. Prenatal fetal hydrops and right heart failure resolved after birth, but progressive left heart failure developed at 5 months. This transition reflects how postnatal hemodynamic changes influence the biventricular noncompaction phenotype, necessitating vigilant monitoring for left heart failure in foetal-onset biventricular noncompaction cases with variants.
Spontaneously resolved pseudoaneurysm after valve-sparing aortic root replacement in Marfan syndrome: a case report - CORRIGENDUM.
Kim EC, Min J, Kwak JG
Cardiol Young · 2026 May · PMID 42083741 · Publisher ↗
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Characteristics and outcomes of patients with occlusion of the left main trunk caused by Kawasaki disease.
Tsuda E
Cardiol Young · 2026 Apr · PMID 42051175 · Publisher ↗
: The outcome of a coronary artery aneurysm at the bifurcation of the left coronary artery caused by Kawasaki disease affects myocardial involvement, which is related to the left ventricular function and outcome of patie...
: The outcome of a coronary artery aneurysm at the bifurcation of the left coronary artery caused by Kawasaki disease affects myocardial involvement, which is related to the left ventricular function and outcome of patients. : A total of eighteen (13 males, 5 females) patients with left main truncus occlusion between 1978 and 2022 were reviewed based on their medical records and selective coronary angiograms. : Most patients had a giant aneurysm at the bifurcation of the left coronary artery. There were two groups based on the timing of the left main trunk occlusion. In the one-time occlusion group, all seven patients had nearly fatal cardiac events. In the second-step occlusion group, 8 (73%) of 11 patients had asymptomatic occlusions of the left main trunk; they underwent grafting to the left anterior descending artery. The median ages at the left main trunk occlusion in the one-time and the second-step group were 18.1 years (range 4.9 to 26.3) and 25.0 years (2.2 to 45.0), respectively. In the second-step group, the median interval from the coronary artery bypass grafting to the left main truncus occlusion was 7.9 years (6 days to 24.5 years). Their left ventricular ejection fraction ranged from 20 to 65% (mean 49%). : To prevent either a fatal cardiac event or a low left ventricular function, a coronary artery bypass graft to the left anterior descending artery was recommended at an optimal time in each patient with a giant aneurysm of the left main trunk complicating a stenotic lesion of the left anterior descending artery.
Outpatient presentation of ventricular fibrillation in paediatric left ventricular assist device patients: a case series with mechanistic insights.
Doğan E, Ülger Tutar Z, Beyter MB … +12 more , Ön ŞŞ, Yılmaz M, Karasulu Beci B, Acar Alkan BB, Kurt H, Levent E, Engin Ç, Yağdı T, Özbaran M, Yazıcı Özkaya P, Tuncer ON, Atay Y
Cardiol Young · 2026 Apr · PMID 42046487 · Publisher ↗
BACKGROUND: Ventricular fibrillation in paediatric recipients of left ventricular assist devices is rare but potentially fatal; however, because systemic perfusion may be temporarily maintained by the mechanical support,...
BACKGROUND: Ventricular fibrillation in paediatric recipients of left ventricular assist devices is rare but potentially fatal; however, because systemic perfusion may be temporarily maintained by the mechanical support, haemodynamic deterioration may be delayed, leading to underrecognition and presentation in the outpatient setting rather than as an immediate in-hospital emergency. METHODS: We report three paediatric left ventricular assist device recipients who developed outpatient malignant ventricular arrhythmias, each representing a distinct mechanistic category: structural, mechanical, and endocrine. Clinical presentation, diagnostic findings, and therapeutic responses were reviewed to highlight pathophysiologic heterogeneity and inform individualised management. RESULTS: The first patient, with biopsy-proven arrhythmogenic right ventricular dysplasia, experienced recurrent ventricular fibrillation refractory to multiple antiarrhythmic agents, ultimately requiring heart transplantation. The second patient presented with ventricular fibrillation secondary to mechanical suction and interventricular septal contact; defibrillation and left ventricular assist device speed reduction restored a stable rhythm with no recurrence. The third patient developed polymorphic ventricular arrhythmias during amiodarone-induced thyrotoxicosis and succumbed to refractory electrical storm and multi-organ failure despite intensive endocrine and antiarrhythmic therapy. CONCLUSIONS: Outpatient ventricular fibrillation in paediatric left ventricular assist device recipients may result from diverse mechanisms, including myocardial structural disease, mechanical pump heart interaction, and systemic endocrine disturbances. Mechanism-guided management combining antiarrhythmic, mechanical, and metabolic interventions is essential for optimal outcomes. Continuous rhythm surveillance and readiness for immediate defibrillation are key to improving survival in this vulnerable group.
A persistent right umbilical vein poses challenges for stent placement in the ductus venosus.
Sakamoto M, Ishii T, Omoto A
Cardiol Young · 2026 Apr · PMID 42046477 · Publisher ↗
We report a case of prenatally diagnosed asplenia syndrome complicated by an infracardiac type total anomalous pulmonary venous connection. Stenting the ductus venosus through the umbilical vein was unsuccessful owing to...
We report a case of prenatally diagnosed asplenia syndrome complicated by an infracardiac type total anomalous pulmonary venous connection. Stenting the ductus venosus through the umbilical vein was unsuccessful owing to the persistence of the right umbilical vein. Umbilical veins can develop various anomalies. When the fetus is planned for catheter intervention immediately after birth, the umbilical vein structure should be evaluated at the prenatal stage.
Predictors of bulboventricular foramen restriction in patients with a dominant single left ventricle and ventriculoarterial discordance.
Ergün S, Tüzün B, Recep E … +6 more , Çoban Ş, Sağlam S, Şahin Işılay İ, Kutanis K, Öztürk E, Hatemi AC
Cardiol Young · 2026 Apr · PMID 42037311 · Publisher ↗
OBJECTIVES: Early identification of patients at risk for bulboventricular foramen restriction is critical for selecting the most appropriate palliative strategy and preventing late reinterventions. The primary aim of our...
OBJECTIVES: Early identification of patients at risk for bulboventricular foramen restriction is critical for selecting the most appropriate palliative strategy and preventing late reinterventions. The primary aim of our study was to identify parameters that predict bulboventricular foramen restriction in this patient population. METHODS: We retrospectively reviewed 17 patients with double-inlet left ventricle or tricuspid atresia associated with ventriculoarterial discordance who underwent staged palliation at our institution between August 2020 and December 2025. The bulboventricular foramen-to-aortic annulus ratio was measured by echocardiography, and receiver operating characteristic analysis was performed to identify a predictive cut-off. RESULTS: Bulboventricular foramen restriction developed in 8 patients (47.1%). Bulboventricular foramen restriction occurred more frequently after pulmonary artery banding with concomitant aortic arch reconstruction (PAB + AR) than after other initial strategies ( = 0.041). The pre-bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio was significantly lower in patients who developed bulboventricular foramen restriction (0.6 [IQR 0.5-0.8] versus 0.8 [IQR 0.67-0,99], = 0.02). Receiver operating characteristic analysis showed that a pre-bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio ≤ 0.65 predicted bulboventricular foramen restriction with an aortic annulus ratio of 0.87 (sensitivity 71%, specificity 83%). In multivariable. CONCLUSIONS: Pulmonary artery banding + arch reconstruction and a pre-bidirectional cavopulmonary shunt bulboventricular foramen/aortic annulus ratio ≤ 0.65 are associated with subsequent bulboventricular foramen restriction. In high-risk patients, performing a Damus-Kaye-Stansel anastomosis at the time of bidirectional cavopulmonary shunt may reduce the need for bulboventricular foramen enlargement and its related complications.
Electrocardiographic findings in paediatric patients with rheumatic diseases using hydroxychloroquine.
Araujo AL, Araujo Júnior E, Rufino IS … +4 more , Fonseca AR, Félix MC, Sztajnbok FR, Bravo-Valenzuela NJ
Cardiol Young · 2026 Apr · PMID 42023741 · Publisher ↗
OBJECTIVE: To evaluate the prevalence and clinical implications of QT interval prolongation and other electrocardiographic changes in paediatric patients with rheumatic diseases using hydroxychloroquine. METHODS: This wa...
OBJECTIVE: To evaluate the prevalence and clinical implications of QT interval prolongation and other electrocardiographic changes in paediatric patients with rheumatic diseases using hydroxychloroquine. METHODS: This was a retrospective and prospective, observational, and analytical study conducted at a centre of perinatology and paediatrics in Rio de Janeiro, Brazil. A total of 26 evaluations of patients ≤18 years old on hydroxychloroquine were included, all following paediatric rheumatology and cardiology services. Patients were included if they had been receiving hydroxychloroquine for at least six months and had complete clinical records; those with pre-existing cardiac conditions unrelated to hydroxychloroquine were excluded. Clinical, demographic, and electrocardiographic data were collected from medical records using standardised protocols. RESULTS: The corrected QT interval was manually measured on 12-lead electrocardiograms and analysed in relation to cumulative drug dose. All electrocardiograms were reviewed independently by two cardiologists to ensure accuracy of corrected QT interval measurements, and discrepancies were resolved by consensus. Most patients were female (76.9%), and systemic lupus erythematosus was the most prevalent diagnosis (88.9%). The cumulative hydroxychloroquine dose ranged from 12 to 447.6 g (mean: 223 g). Corrected QT interval values ranged from 377 to 454 ms (mean: 413 ms). Correlation analysis between cumulative dose and corrected QT interval showed a weak negative association (r = -0.24; p = 0.338), not statistically significant. Simple linear regression confirmed no association between variables (R = 5.7%). CONCLUSION: In this cohort of paediatric patients with rheumatic diseases, no significant association was observed between cumulative hydroxychloroquine use and QT interval prolongation.
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