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Background: Single ventricle (SV) patients with a Fontan circulation are at risk for functional deterioration. The aim of this study was to assess longitudinal Fontan hemodynamics using serial cardiovascular magnetic resonance (CMR) data and to study the impact of aortopulmonary collateral (APC) flow and type of SV morphology., Methods: Forty-one Fontan patients (age at first CMR 13.4 ± 6.0 years) with two CMR examinations within a time interval of at least 4 years (follow-up 5.3 ± 0.9 years) were included. The protocol consisted of short-axis cine volumetry and 2-dimensional blood flow measurements in the inferior vena cava (IVC), superior vena cava (SVC) and ascending aorta (Ao). APC flow was calculated as Ao - (SVC + IVC). Myocardial strain/strain rate was assessed using feature tracking technique., Results: SV end-diastolic volume (p = 0.14) and ejection fraction (p = 0.70) remained constant. No significant changes in CMR derived myocardial strain/strain rate values were observed. Ao flow decreased (p = 0.01), IVC flow remained unchanged (p = 0.52), while SVC flow (p = 0.003) and APC flow (p = 0.006) decreased significantly. Patients with a systemic right ventricle (RV) showed unchanged APC flow and a further increase in SV size over time compared to patients with a systemic left ventricle., Conclusions: Longitudinal CMR data in a cohort of clinically stable Fontan patients revealed no significant changes in SV dimensions and myocardial performance while APC flow decreased spontaneously. Patients with a systemic RV seem to be at risk for permanent SV volume overload through APCs and might therefore benefit from consequent interventional APC embolization., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: The long-term impact of altered hemodynamics after stage II in children with hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC) on cerebral growth and neurodevelopmental outcome is unknown. We aimed to investigate whether elevated central venous and atrial filling pressures before the Fontan procedure may be associated with smaller brain volumes and poorer neurodevelopmental outcome after the initial hybrid procedure., Methods: In a two-center cohort study semiautomated segmentation of cerebral magnetic resonance imaging scans was conducted in 25 children with HLHS/HLHC (25 hybrid) before the Fontan procedure (27.6 ± 4.3 months) and in 8 healthy control subjects (29.7 ± 9.5 months). Study patients were evaluated with the Bayley Scales of Infant and Toddler Development III (Bayley-III) and a neurologic examination. Hemodynamic measures after stage II were assessed with cardiac catheterization at 2 years of age before Fontan completion. Children with known genetic comorbidities were excluded., Results: In HLHS/HLHC patients higher atrial filling pressures (6 ± 3 mm Hg; range, 2-14) were correlated with reduced brain volumes and lower language composite score, whereas higher Glenn pressures (10 ± 3 mm Hg; range, 6-16) were related to higher cerebrospinal fluid, reduced brain volumes, and lower cognitive, language, and motoric composite scores in the Bayley-III. Compared with control subjects white matter volumes were reduced and cerebrospinal fluid volumes increased in study patients., Conclusions: These data suggest that altered cardiovascular hemodynamics after stage II influence brain growth and neurodevelopmental outcome in infants with HLHS/HLHC., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The surgical creation of a Potts shunt has been reported in children with suprasystemic idiopathic pulmonary artery (PA) hypertension (IPAH) refractory to any medical therapy. This surgical approach allows acute decompression of the right ventricle (RV) and thereby avoids ventricular failure. We present 4 newborns and infants with different causes of pulmonary hypertension (PH) in whom interventional stenting of the patent ductus arteriosus (PDA) was used to create a "functional" Potts shunt., Methods: Suprasystemic PH was diagnosed by cardiac catheterization in 2 newborns with complex left heart obstructive lesions, a patient with persistent PH of the newborn (PPHN), and an infant with IPAH and was accompanied by RV dilation and imminent RV failure., Results: Stenting of the PDA was performed uneventfully during cardiac catheterization in all patients and led to stabilization of clinical symptoms. The 2 patients with complex cardiac lesions subsequently underwent successful biventricular repair. In the PPHN patient, the stented PDA was closed interventionally when PA pressures dropped and a significant left-to-right shunt occurred. PA pressures in the patient with IPAH remained high while the stented PDA still served as RV decompression in avoidance of lung transplantation., Conclusions: Newborns and infants with suprasystemic PH of varying etiologies may benefit from the creation of a functional Potts shunt by stenting the PDA. This strategy should be considered in patients with suprasystemic IPAH or PPHN as a temporary or permanent therapy when a PDA can be identified at cardiac catheterization. It may also be beneficial in patients with PH due to left heart obstructions by serving as a bridge to further corrective surgery., (Copyright © 2014 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: In patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon. Using late gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) imaging, myocardial scarring has been shown in ALCAPA patients late after repair, however the incidence of scarring before surgery and its impact on postoperative course after surgical repair remained unknown., Methods: 8 ALCAPA-patients (mean age 10.0 ± 5.8 months) underwent CMR before and early after (mean 4.9 ± 2.5 months) coronary reimplantation procedures. CMR included functional analysis and LGE for detection of myocardial scars., Results: LV dilatation (mean LVEDVI 171 ± 94 ml/m2) and dysfunction (mean LV-EF 22 ± 10 %) was present in all patients and improved significantly after surgery (mean LVEDV 68 ± 42 ml/m2, p=0.02; mean LV-EF 58 ± 19 %, p<0.001). Preoperative CMR revealed myocardial scarring in 2 of the 8 patients and did not predict postoperative course. At follow-up CMR, one LGE-positive patient showed delayed recovery of LV function while myocardial scarring was still present in both patients. In two patients new-onset transmural scarring was found, although functional recovery after operation was sufficient. One of them showed a stenosis of the left coronary artery and required resurgery., Conclusions: Despite diminished myocardial perfusion and severely compromised LV function, myocardial scarring was preoperatively only infrequently present. Improvement of myocardial function was independent of new-onset scarring while the impact of preoperative scarring still needs to be defined.

We report a 20-year-old patient with Shone's complex and severe diastolic dysfunction of his small left ventricle (LV) in whom severe pulmonary hypertension and biventricular failure developed while he was awaiting combined heart-lung transplantation. We performed a percutaneous balloon atrial septostomy and a modified Potts shunt (13-mm graft from left pulmonary artery to descending aorta), with the aim of decompressing the hypertensive right ventricle (RV) by reducing left ventricular preload and left atrial hypertension. The procedures were uneventful. The patient's condition improved rapidly and biventricular function was restored. In contrast to a Potts shunt in other conditions, patients with pulmonary hypertension caused by left ventricular disease may benefit from an additional atrial left-to-right shunt., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Dilated cardiomyopathy (DCM) in childhood has a considerable morbidity and mortality and high incidence of heart transplantation. Pulmonary artery banding (PAB) has been proposed in patients with corrected transposition of the great arteries to retrain the sub-pulmonic left ventricle (LV) and to improve a failing sub-aortic right ventricle. We evaluated the short-term and medium-term effects of PAB in young patients with LVDCM., Methods: A retrospective single-center observational study was performed to evaluate the possible benefits of a dilatable surgical PAB in infants and young children with LVDCM., Results: Reported are 12 patients (10 infants, 2 toddlers) with LVDCM referred for heart transplant who received a surgical PAB. There were no hospital deaths. Clinical functional status improved in all patients. The pressure gradient across the PAB increased within 20 days from 28 ± 7 to 43 ± 15 mm Hg. The LV ejection fraction increased from 14.5% ± 5% pre-PAB to 27% ± 13% at hospital discharge and to 47% ± 10% at 3 to 6 months. The LV end-diastolic diameter (z-score) decreased (p > 0.001) from 46 ± 6.1 (+7.0 ± 1.3) to 35 ± 15 mm (+3.0 ± 1.3) after 3 to 6 months and to 34 ± 15 mm (+1.3 ± 1.14) after a median age of 2 years (maximum 6.6 years), respectively. Plasma B-type natriuretic peptide levels decreased from 3431 ± 2610 to 288 ± 321 pg/ml at discharge and to 102 ± 96 pg/ml 22 months later. Eight children were subsequently de-banded by transcatheter technique and 6 of them are currently at Ross Heart Failure Classification for Children class I. Two patients, both with non-compaction DCM, deteriorated at 5 and 6 months after PAB debanding and finally died., Conclusion: In young children with LVDCM and still-preserved right ventricular function, PAB led to an improvement of LV and mitral valve function by ventricular interaction., (Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Novel surgical strategies have dramatically improved the initial outcome of newborns with hypoplastic left heart syndrome. However, the single systemic right ventricle remains a major challenge, with limited effectiveness of pharmacologic therapy. The present case documents that the intracoronary administration of autologous bone marrow-derived progenitor cells is technically feasible in a critically ill infant with hypoplastic left heart syndrome and severe heart failure after a hybrid comprehensive stage II procedure. Cell therapy might represent an option before heart transplantation in children with single ventricle physiology presenting with severe heart failure., (Copyright (c) 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)