Markus Khalil

Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.

Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH.

Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review

Objective A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition. Methods A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations. Published clinical Fontan studies that were written in English and included at least four patients with PLE were selected. PLE definitions were quantitatively analysed using a lateral thinking tool in which definitions were fractionated into constituent pieces of information (building blocks or diagnostic criteria). Results We identified 364 papers. In the final analysis, data from 62 published articles were extracted. A diagnostic definition of PLE was used in only 27/62 (43.5%) of selected studies, and definitions were very heterogeneous. We identified eight major diagnostic criteria. Hypoalbuminaemia (n=23 studies, 85.2%), clinical presentation (n=18, 66.7%), documentation of enteric protein loss (n=16, 59.3%) and exclusion of other causes of hypoproteinaemia (n=17, 63.0%), were the most frequently used diagnostic criteria. Most studies used three diagnostic variables (n=13/27, 48.1%). Cut-off values for laboratory parameters (serum albumin, protein or faecal α-1-antitrypsin) were frequently incorporated in the PLE definition (n=16, 59.3%). Conclusions Establishment of a universally accepted PLE definition for routine use in clinical research and daily practice is required. The diagnostic criteria may help constitute a diagnostic PLE definition.

Percutaneous pulmonary valve implantation for reconstruction of a patch‐repaired right ventricular outflow tract

Percutaneous pulmonary valve implantation (PPVI) is nowadays an accepted treatment option to repair post-surgical conduit dysfunction of the right ventricular outflow tract (RVOT). In addition, many patients need a pulmonary valve to reconstruct a hemodynamically incompetent native or conduit free outflow tract. Based on our experience with percutaneous stent-valve placement in a cohort of 125 patients, we report here transvenous reconstruction of a conduit-free, patch repaired outflow tract by utilizing balloon-expandable stent-valves in 23 patients with a median age of 22 years (5-60 years). In 20 patients, the step-by-step procedure was performed uneventful with the aimed success. Severe RVOT dysfunction in term of a clinical relevant regurgitation could be changed to mild, as it was confirmed by follow-up color Doppler echocardiography. In a 5-year-old girl a Melody® valve was placed as a surgical-interventional hybrid approach. In one patient, the procedure was complicated by stent embolization during preparation of the RVOT for stent-valve implantation. Reposition of the embolized stent was nevertheless successful for finishing percutaneous valve-implantation. In one patient, surgical approach became necessary because of the inability to advance the balloon-mounted stent-valve through a pre-stented RVOT. Considering the current available balloon-expandable stent-valves, transvenous pulmonary valve implantation is feasible to treat even an incompetent conduit-free RVOT. However, preparation of the RVOT by pre-stenting, in most patients with more than two stents in telescope technique remains challenging. Reconstruction of RVOT by the current available valves is promising only for a carefully selected group of patients.

Creation of a restrictive atrial communication in heart failure with preserved and mid-range ejection fraction: effective palliation of left atrial hypertension and pulmonary congestion

BackgroundLeft atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect.Methods and resultsFrom June 2009 to December 2016, 24 patients (50% with an age less than 16xa0years) with HFpEF were palliated; NYHA-/Ross class IV (nu2009=u200910); median systemic ventricular ejection fraction 64 (range 35–78) %. Cardiomyopathy was classified as a restrictive (nu2009=u20094) or hypertrophic (nu2009=u20092). (75% related to congenital heart defects) Three patients had a systemic right ventricle; in the majority of patients, HFpEF was associated to complex congenital heart defects (nu2009=u200918). Mean pulmonary arterial pressures (PAP systolic/diastolic) were 56/28 (±u200924/13), left atrial pressures (LAP, v-, a-wave, mean) 26/25/20 (±u20097/10/6). Trans-septal puncture was used in 22 patients; foramen ovale dilatation in 2 patients. Median balloon size was 12 (range 6–18) mm; procedure time including diagnostic measures 125 (83–221) min. No procedural death or complications were observed. Mean LA-pressures decreased significantly to 19/19/15u2009±u20096/8/5xa0mmHg (pu2009=u20090.05); median brain natriuretic peptide (BNP) decreased from 392 (range 93–4401) pg/ml median BNP to 314 (range 61–1544) pg/ml (pu2009=u20090.05). Three patients died; one patient received orthotopic heart and one patient a heart–lung transplantation. No patient required so far an assist device. Clinical improvement occurred in all patients, in some after additional surgical or interventional approach.ConclusionsTranscatheter LA decompression is an age-independent, effective palliation treating patients with HFpEF.

Endocardial Fibroelastosis of the Left Ventricle Affects Right Ventricular Performance in Fetuses with Hypoplastic Left Heart Syndrome: A Prospective Study Using M-Mode, PW- and Tissue Doppler Techniques

PURPOSEnu2002Myocardial function (MF) of the systemic right ventricle (RV) influences the postnatal course of neonates with hypoplastic left heart syndrome (HLHS). Our study examines whether the presence of endocardial fibroelastosis of the left ventricle (LV EFE) influences MF of the RV in HLHS fetuses.nnnMATERIALS AND METHODSnu2002A prospective study was conducted including 10 controls (group 1), 10 HLHS fetuses with (group 2) and 10 without LV EFE (group 3) - all matched for gestational age. M-mode was used to assess tricuspid plane systolic excursion (TAPSE) and the shortening fraction (SF). PW-Doppler-derived and PW-TDI-derived velocities were assessed. E/A, E/e, e/a ratios and the myocardial performance index (mpi) were calculated.nnnRESULTSnu2002The examination of MF revealed significantly lower sxa0velocities (pu200a<u200a0.05) and higher values for SF in group 2 compared to group 3. e/a ratio, et (ejection time), E wave velocity, E/e and SF showed significantly higher values in group 2 compared to group 1. In group 2 a velocity increased significantly over gestational age. In group 3 but not in group 2, TAPSE increased during gestation.nnnCONCLUSIONnu2002These significant differences in MF between the groups might lend support to the notion of negative ventricular-ventricular interaction in the case of HLHS with LV EFE possibly influencing surgical outcomes.

Surgical-Interventional Hybrid Concept in a Newborn With Borderline Left Heart

Through a series of complex surgical and interventional procedures including downsizing of an unrestricted atrial septal defect, aortic arch reconstruction, pulmonary artery banding, reversed Potts shunt, and Melody valve in a mitral position, biventricular conversion was successfully achieved in a patient with a distinct borderline left ventricle. By use of these interventional steps, flow-mediated and load-mediated growth of a small left ventricle was observed, highlighting the plasticity of hearts in growing children. Surgical and catheter interventional teamwork may lead to fascinating results, provoking us to quote Aristotles wisdom: The whole is more than the sum of its parts.

Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure

Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median ageu2009=u20094.3 years; rangeu2009=u200933 days–26 years; median body weightu2009=u200914u2009kg; rangeu2009=u20093–71u2009kg; NYHA-/Ross class IV; nu2009=u200911). PAH was classified as idiopathic (nu2009=u20096) or secondary (nu2009=u20095). History of syncope was dominant (nu2009=u20096); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10u2009mm (rangeu2009=u20096–14u2009mm); the mean catheter time was 174.6u2009±u200948u2009min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97u2009±u20092 to 89u2009±u200911.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.

Perinatal outcomes of congenital heart disease after emergent neonatal cardiac procedures

Abstract Purpose: We compared outcome of neonates with prenatal and post-natal diagnosis of congenital heart disease presenting in our paediatric heart centre between March 2005 and May 2015 who underwent an emergent intervention within 48u2009h post-partum. Materials and methods: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized foetal echocardiography, diagnosis was made post-natally. In 98/111 (88.2%), 30-day outcome was known. Results: Regarding the entire cohort, 30-day survival did not differ significantly in prenatal and post-natal diagnosis group (71.2 vs. 72.9%; pu2009>u2009.1). Infants with prenatal diagnosis were more likely to be born by caesarean section (59.6% vs. 33.9%, pu2009=u2009.01). Those with post-natal diagnosis had a higher need for intubation (32.7% vs. 52.5%; pu2009<u2009.05). Subgroup analysis of HLH/HLHC (hypoplastic left heart/hypoplastic left heart complex) patients revealed higher number of deaths within 30 days of life in the post-natal diagnosis group, although the difference did not reach statistical significance (5/7, 71.4% vs. 5/20, 25.0%; pu2009=u2009.075). Conclusion: For newborns who require emergent neonatal cardiac procedures, our results point towards a lower death rate after prenatal diagnosis of HLH/HLHC.

Clinical and prognostic value of endothelin-1 and big endothelin-1 expression in children with pulmonary hypertension

Objective Pulmonary arterial hypertension is known to be associated with increased expression of endothelin (ET)-1 and its precursor big ET-1. Therefore, we hypothesised that in children with pulmonary hypertension (PH) altered levels of ET-1 and big ET-1 may have clinical and prognostic impact. Methods Sixty-six children with different forms of PH (mean age 10.4±9.7u2005years) were included. Blood samples were taken from the pulmonary artery and a systemic artery. Levels of ET-1/big ET-1 were measured via ELISA method and compared with clinical and haemodynamic data. To assess prognostic relevance, Kaplan–Meier survival analysis was conducted with definition of end point as the composite of mortality, lung transplantation, use of intravenous prostanoids and Potts shunt creation. Results ET-1 levels ranged between 0.09 and 11.64 (mean 1.48±2.34) fmol/mL, and big ET-1 levels between 0.05 and 2.92 (mean 0.84±0.58) fmol/mL. No significant relationships were found between ET-1/big ET-1 levels and functional class as well as haemodynamic indices of PH severity. Mean follow-up after catheterisation was 63.2±44.1u2005months. While 31 of the 66 (47%) patients with PH reached a predefined end point, there was no significant relation between levels of ET-1/big ET-1 and patient outcome. Conclusions Although children with PH had alterations in ET-1/big ET-1 expression, which may reflect changes in net release or lung clearance, levels of ET-1/big ET-1 showed no correlation with clinical and haemodynamic parameters, and were not able to predict outcome.