Tricuspid Annular Plane Systolic Excursion (TAPSE) correlates with mean pulmonary artery pressure especially 10 years after pediatric heart transplantation.

Tricuspid annular plane systolic excursion (TAPSE) is important in the noninvasive echocardiographic assessment of right heart function. This retrospective observational study shows correlations of TAPSE with invasive right heart catheterization parameters after pediatric heart transplantation (HTx). The study included patients after pediatric HTx with cardiac catheterizations in 2018/2019 and measurement of TAPSE (n = 52 patients with 57 examinations; 50.9% adults, 52.6% female, median age: 18.54 years). TAPSE was compared with normal values. Stepwise, linear and multiple regression were used to show influencing variables on TAPSE. Mean TAPSE z-score was -3.48 (SD: 2.25) and 68.4% of HTx-recipients showed abnormally reduced TAPSE (z-score ←2) compared to normal values. Multiple regression (p-value <0.001; corrected R2 = 0.338) showed significant correlations of time since HTx (p-value <0.001) and mPAP (p-value: 0.008) with TAPSE z-scores. Divided into subgroups (time since HTx <10 and ≥10 years), TAPSE and mPAP correlated only ≥10 years after HTx (p-value = 0.002). This study provides data of TAPSE even ≥10 years after pediatric HTx. Most patients showed a decreased TAPSE early after HTx, which improved over time. TAPSE z-scores correlated significantly with time since HTx and mPAP, especially ≥10 years post-HTx. Therefore, TAPSE must be used carefully in the early follow-up.

Conversion to everolimus in pediatric heart transplant recipients is a safe treatment option with an impact on cardiac allograft vasculopathy and renal function.

BACKGROUND: Cardiac allograft vasculopathy (CAV) and nephrotoxicity affect long-term survival after heart transplantation (HTX). Studies, mostly conducted in adults, showed a positive effect of everolimus (EVL) on these problems. We describe the effects of conversion of the immunosuppressive therapy to an everolimus including regime on CAV, renal function, and safety in heart transplanted children/adolescents. METHODS: This retrospective single-center study included 36 participants (mean time after HTX 6.3 ± 4.7 years). Descriptive pre/post-comparisons were performed with an observation period partially up to 4 years. Impact on CAV was assessed based on intravascular imaging and Stanford grading. Safety analysis included cytomegalovirus (CMV)-infection and acute rejection. RESULTS: In terms of CAV (9 out of 36 patients) four showed no progression, three an improvement, one a worsening; one new diagnosis. The average CrCl showed a significant improvement 6, 12, and 24 months after conversion regarding all patients (n = 29). There was no acute rejection or CMV-infection. CONCLUSION: Conversion to an EVL-based therapy after pediatric HTX is a safe immunosuppressive regime without increasing risk of acute rejection or CMV-infection. There was some evidence of reduction in progression of CAV and a significant improvement of the renal function.

Percutaneous pulmonary valve implantation in a dysfunctional Trifecta® bioprothesis after high-pressure balloon fracturing.

A percutaneous pulmonary valve-in-valve (PPVIV) implantation in small surgical tissue valves may be limited due to the valve's initial diameter. Fracturing of the valve's integrity by high-pressure balloons may enhance the diameter and facilitate subsequent PPVIV with a large valve. To the best of our knowledge, the Trifecta® valve seemed not to be accessible for fracturing. We report a case of successful 19-mm Trifecta valve fracturing, followed by PPVIV using a 26-mm Edwards SAPIEN 3 valve in pulmonary position. By repetitively using a high-pressure balloon 5 mm larger than the labeled valve size, we were able to fracture the valve's integrity and implant a 26-mm valve thereafter. Therefore, Trifecta valve appears to be suitable for valve ring fracturing and subsequent PPVIV in certain patients.

Motor outcome, executive functioning, and health-related quality of life of children, adolescents, and young adults after ventricular assist device and heart transplantation.

OBJECTIVE: The aim of the current study is to measure long-term executive function, motor outcome, and QoL in children, adolescents, and young adults after VAD and Htx. METHODS: Patients were examined during routine follow-up. Investigation tools were used as follows: Examination for MND of motor outcomes, Epitrack® for attention and executive functioning, and Kidscreen-52 and EQ-5D-5L questionnaires for QoL. Additional data were retrospectively obtained by an analysis of patient medical records. RESULTS: Out of 145 heart transplant recipients at the department of pediatric cardiology of the University Hospital Munich, 39 were implanted with a VAD between 1992 and 2016. Seventeen (43.6%) patients died before or after Htx; 22 (56.4%) patients were included in our study. Mean age at transplant was 9.52 years (range: 0.58-24.39 years, median 9), and the mean follow-up time after Htx was 6.18 years (range: 0.05-14.60 years, median 5.82). MND examination could be performed in 13 patients (normal MND: n = 11, simple MND: n = 1, complex MND: n = 1). Executive functioning was tested in 15 patients. Two (13.3%) patients had good results, six (40%) average results, three (20%) borderline results, and four (26.7%) impaired results. QoL (Kidscreen n = 7, EQ-5D-5L n = 8) was similar to a healthy German population. CONCLUSION: Motor outcome, executive functioning and QoL in survivors of VAD bridging therapy and Htx can be good, though underlying diseases and therapies are associated with a high risk of cerebral ischemic or hemorrhagic complications.

Von Willebrand factor parameters as potential biomarkers for disease activity and coronary artery lesion in patients with Kawasaki disease.

Elevated von Willebrand factor (vWF):Antigen plasma levels have been observed in conjunction with cardiovascular diseases or vasculitis. The association of Kawasaki disease, a vascular inflammatory disease and vWF:Antigen, vWF:Collagen binding activity, and vWF multimers is unknown. We therefore investigated vWF parameters in 28 patients with acute Kawasaki disease in association with disease activity and coronary artery lesions. VWF:Antigen and vWF:Collagen binding activity were assessed via enzyme-linked immunoassay. The ratio of both (vWF:Collagen binding activity and VWF:Antigen) was calculated and vWF multimeric structure analysis performed. We analyzed the association between vWF parameters and our clinical data focusing on coronary artery outcome. VWF:Antigen and vWF:Collagen binding activity levels were significantly higher in the acute than in the disease's convalescence phase, and correlated positively with CRP levels. Neither variable was associated with coronary artery lesions. The vWF:Collagen binding activity/vWF:Antigen ratio, however, was significantly decreased in patients with a coronary artery lesion (z-score > 2; N = 10; mean ratio 0.96 vs. 0.64; p = 0.031) and even more so in those with a coronary artery aneurysm (z-score > 2.5; N = 8; mean ratio 0.94 vs. 0.55; p = 0.02). In a sub-analysis, those patients with a very low ratio in the acute phase presented a persistent coronary artery aneurysm at their 1-year follow-up.Conclusion: This study suggests that comprehensive analysis of vWF parameters may help to both monitor KD inflammation and facilitate the identification of those patients carrying an increased risk for coronary artery lesion.What is Known:• Von Willebrand factor (VWF)-parameters represent surrogate markers for vascular inflammation.• Kawasaki disease is a generalized vasculitis in children, which can be complicated by coronary artery lesions.What is New:• In those Kawasaki disease patients with coronary artery lesions, the vWF:CB/vWF:Ag ratio was significantly decreased.• VWF parameters may help to identify patients at risk for coronary artery lesions.

Edwards Sapien 3 transcatheter aortic valve implantation for management of severe aortic regurgitation in a teenage patient with corrected atrioventricular septal defect and progressive left ventricular dysfunction.

Transcatheter aortic valve replacement is mostly performed in elderly patients with severely calcified aortic valves. There are few reports about its use for pure aortic regurgitation, few reports about its use in adolescent patients and to the best of our knowledge no report about the use of an Edwards Sapien valve in the aortic position in an underage patient after surgically corrected congenital heart disease (CHD). Decompensation of a complex CHD can be difficult to manage and may require unusual solutions. We report a case of a teenage patient presenting with progressive aortic regurgitation and deterioration of left ventricular function after multiple surgical procedures for an atrioventricular septal defect (AVSD). As "bridge-to-transplant," we performed a transcatheter aortic valve implantation using a balloon-expandable Sapien 3 prosthesis. At 6 month follow-up, the patient remained clinically stable with no rehospitalization due to heart failure.

The Creation of an Interatrial Right-To-Left Shunt in Patients with Severe, Irreversible Pulmonary Hypertension: Rationale, Devices, Outcomes.

PURPOSE OF THE REVIEW: Targeted pharmacotherapies did improve survival rates, exercise capacity, and quality of life (QoL) of PAH patients. However, these pharmacological interventions are expensive and not always accessible. In addition, not all patients do respond similarly to these medications and many will continue to deteriorate. This review aims to discuss the beneficial role of an artificial right-to-left shunt and highlights current interventional devices and outcomes. RECENT FINDINGS: Since patients with preexisting atrial shunts or patients with Eisenmenger's disease show better survival rates, improved exercise capacity, and QoL, PAH patients clinically do benefit from an atrial septostomy by reducing signs of right heart failure, improving left heart filling, cardiac output, and systemic oxygen transport despite hypoxia. However, an uncontrolled septostomy with unrestricted right-to-left shunt bears the risk of acute severe desaturation and death. The Atrial Flow Regulator (AFR device, Occlutech®, Sweden) provides an adjustable shunt size with restricted flow and excellent short-term outcomes. Interventional strategies for PAH palliation are on the rise. The novel AFR device provides a durable and safe option for a controlled right-to-left shunting, thus enabling an individualized management.

Edwards Sapien XT® pulmonic valve compression after resuscitation and successful redilatation.

Despite the increasing use of percutaenous valves, little is known about valve performance after external chest compression. We report a case of a compressed pulmonary Edwards Sapien® XT valve after resuscitation. With the patient on ECMO, successful redilatation was performed with unimpaired postprocedural valve function. We aim to increase the awareness for valve distortion after external chest compression with increasing numbers of transcatheter valvulations. We suggest that immediate re-evaluation of the implanted device via biplane fluoroscopy is mandatory after resuscitation in such cases.

Creation of a defined and stable Fontan fenestration with the new Occlutech Atrial Flow Regulator (AFR®).

This report describes the first clinical experience with the new Occlutech Atrial Flow Regulator (AFR®) device for implementation of a late Fontan fenestration. The AFR® device secured a defined diameter of the fenestration without spontaneous re-occlusion of this extra-anatomic perforation. This ensured a permanent clinical improvement of our failing Fontan patient.

Closure of very large PDA with pulmonary hypertension: Initial clinical case-series with the new Occlutech® PDA occluder.

OBJECTIVE: We report our recent experience with the new Occlutech® PDA Occluder, which seems suitable especially for closure of large patent ductus arteriosus (PDA) sizes with elevated pulmonary artery pressure (PAH). BACKGROUND: Although considered a standard procedure in pediatric cath labs, closure of very large PDA (PDA/Aorta ratio > 0.5) and PAH remains a challenge. METHODS/RESULTS: The Occlutech® PDA occluder was used in eight children and adolescents (age 4-16, median 10.75 years), body weight from 14 to 54 kg (median 21 kg) with very large PDA and PAH: PDA diameter was 5-13 mm (median 10 mm); PAP/Aortic pressure ratio was 0.58-0.96. Sufficient occlusion of the PDA was documented by angiography and/or echocardiography in all cases. Four different device sizes were selected: Minimal diameter 6-14 mm (median 12 mm), length 6.3-16 mm (median 14 mm), aortic disc 13-24 mm (median 20 mm), size at the pulmonary end 8-18 mm (median 15 mm). A balloon test occlusion of the PDA was performed in five patients in order to evaulate the decline of pulmonary artery pressure or to delineate the exact anatomy of the PDA. Before release of the device, a careful "wiggle manoevre" was performed to assess the stability of the implanted device and to proof the inability to embolize to the aorta. CONCLUSIONS: With the new Occlutech® PDA Occluder closure of very large PDAs and PAH is feasible and efficient. The wider pulmonary artery end of the Occlutech® PDA Occluder offers enhanced stability and reduces the risk of embolization. © 2016 Wiley Periodicals, Inc.

The "Dog Bone Technique" A Novel Easy and Safe Catheter Maneuver for Aortic Arch and Coarctation Stenting.

BACKGROUND: Various techniques are described to facilitate stable stent implantation in aortic arch stenosis or coarctation. We describe an alternative technique, which due to its unique appearance during stent implantation, we have named "Dog Bone Technique" (DBT). TECHNIQUE: The stent/balloon assembly is placed across the stenosis, the long sheath is retrieved to uncover the distal 20-50% of the stent. The balloon is inflated with the pressure inflator just to expand slightly the stent. Thereafter the sheath is pulled back and the proximal end is uncovered and partially inflated; therewith the assembly takes the typical "dog bone" shape before complete inflation and final positioning. Repositioning of the stent and control angiography is possible at each time of this procedure. RESULTS: Between 1/2010 and 12/2014 we implanted 91 stents in 87 patients (mean age 20.2 years). About 71 patients had native or re-coarctation and 16 patients had transverse aortic arch stenosis. In 38 patients (44%) a pharmacological exercise test with Orciprenaline was performed during implantation resulting in high-cardiac output. In none of the patients reduction of cardiac output by adenosine or a rapid pacing of the right ventricle was required for stable stent implantation. All stents were implanted in the targeted position using this single balloon technique. There were no acute or short-term complications detected. CONCLUSION: DBT is a safe and feasible technique for aortic stent implantation even at high-cardiac output. Other additional techniques for stent placement are not necessary to obtain a stable final position in the target region.

Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis. Medical records were retrospectively reviewed. The hospital survival was 100%. In EC group, cardiopulmonary bypass time (CPB) was shorter (p = 0.004) and less patients needed aortic cross-clamping (p < 0.001). Children after EC stayed longer in the hospital (p = 0.016) and manifested more often prolonged effusions (p = 0.038). The incidence of all forms of junctional rhythm was higher in the LT group, early postoperatively (p < 0.001), during hospitalization (p = 0.004) and at discharge (p < 0.001). Children after LT required more often temporary pacemaker stimulation (p < 0.001). Patients without postoperative normofrequent sinus rhythm had longer CPB time (p = 0.008) and were more often operated on with aortic cross-clamping (p = 0.028). Lateral atrial tunnel Fontan operation with fenestration facilitates early adaptation to the total passive pulmonary flow, but predisposes the patients to the loss of sinus rhythm. The crucial role in the preservation of sinus rhythm plays the last step of the multistage surgery of the single ventricle malformations, probably not only the surgical technique but also factors associated with the cardiopulmonary bypass.

Comparison of the Prolonged- and Immediate-Release Tacrolimus Capsule Formulation: The Patient's View and Medication Satisfaction of Patients After Pediatric Heart Transplantation.

BACKGROUND: Medication adherence is essential for long-term success after pediatric organ transplantation. Causes of reduced adherence should be detected early to improve the consequent medication intake. We describe the influence of switching from tacrolimus twice daily (tacrolimus-BID) to tacrolimus once daily (tacrolimus-QD) on medication satisfaction and medication adherence in patients after pediatric heart transplantation. METHODS: A retrospective analysis was conducted regarding patient satisfaction and adherence to the immunosuppressant tacrolimus after pediatric heart transplantation, before and after conversion from tacrolimus-BID to tacrolimus-QD, using questionnaires. RESULTS: Thirty-eight patients were enrolled (tacrolimus-BID: n = 35, mean age 15.7 ± 5.2 years; tacrolimus-QD: n = 38, mean age 16.2 ± 5.6 years). The amount of unadministered medication in the last 3 months did not differ between the 2 pharmaceutical forms. However, 17% (n = 6) reported unstable tacrolimus trough levels when taking tacrolimus-BID, vs 8% (n = 3) under tacrolimus-QD (P = .453). However, there was no statistically significant difference in the stability of the last 6 trough levels of each patient (P = .074). A total of 57% (n = 20) of patients had subjective side effects before conversion, compared to only 29% (n = 11) after conversion (P = .013). Regarding the intensity of the side effects, 6 patients reported strong/very strong side effects when taking tacrolimus-BID vs 1 patient when taking tacrolimus-QD (P = .250). In addition, the overall satisfaction with the immunosuppressant was higher under tacrolimus-QD (92% vs 83%; P = .508). However, this improvement was statistically not significant and may not be clinically relevant. CONCLUSIONS: The amount of forgotten medication was not reduced after conversion from tacrolimus-BID to tacrolimus-QD. However, subjective side effects as well as patient satisfaction improved under tacrolimus-QD.

An unusual form of supracardiac total anomalous pulmonary venous return via a right-sided vertical vein in a heterotaxy syndrome case.

Heterotaxy syndromes comprise a great variety of possible cardiac defects. Anomalies of the pulmonary venous system are well recognized in heterotaxy syndromes but constitute a frequently underdiagnosed subgroup. The case report describes a girl with a rare form of supracardiac total anomalous pulmonary venous return via a right-sided vertical vein to the right innominate vein in heterotaxy syndrome with dextrocardia, unbalanced atrioventricular septal defect with severe left ventricular hypoplasia, and transposition of the great arteries with pulmonary stenosis. Careful evaluation and imaging before surgical repair is crucial for such complex cardiac anomalies.

Cobra-Head Cuffed Vascular Graft as Right Ventricle-to-Pulmonary Artery Shunt in Norwood Procedure.

BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) shunt as a part of the Norwood procedure underwent many modifications. We present our experience with a commercially available polytetrafluoroethylene vascular graft with cobra-head cuff as an RV-PA shunt. METHODS: A consecutive series of 52 children with hypoplastic left heart syndrome (median age 8 [range, 2-68] days, median weight 3200 [range, 2060-4400] g) underwent the Norwood procedure with a cobra-head cuffed RV-PA shunt (6 mm). The cuffed end was used for the central PA reconstruction. A retrospective analysis of clinical results, PAs development, and shunt-related complications, interventions, and technique of Glenn operation was performed. The study endpoint was Glenn operation with shunt removal or interstage death. RESULTS: The hospital and late interstage mortality was 3.8% (n = 2 of 52) and 4% (n = 2 of 50), respectively, and was not shunt-related. During mean follow of 3.7 ± 2.5 years, 48 (92.3%) children underwent Glenn operation at a median age of 6 (range, 2.6-9.1) months. Angiography before the second stage revealed satisfactory branch PAs development (maximum and minimum McGoon ratio of 1.95 ± 0.36 and 1.38 ± 0.38, respectively). The mean maximal diameter of the left PA was smaller than that of the right PA (7.13 ± 2.1 mm vs 8.42 ± 2.2 mm; P = .017), without differences in mean minimal diameter. Two infants required stent implantation in proximal shunt end and 1 required urgent Glenn operation because distal shunt thrombosis. During Glenn operation, 11 (22.9%) children required patch reconstruction of central PAs. CONCLUSIONS: The cobra-head cuffed graft allowed easy and reproducible reconstruction of the central PA during the Norwood procedure. Using this technique, the development of PAs is satisfactory, the rate of shunt-related complications and interventions is low, and the second stage can be performed without patch material.

Cardiac allograft vasculopathy: Differences of absolute and relative intimal hyperplasia in children versus adults in optical coherence tomography.

BACKGROUND: Intracoronary imaging enables an early detection of intimal changes. To what extend the development of absolute and relative intimal hyperplasia in intracoronary imaging differs depending on age and post-transplant time is not known. METHODS: Aim of our retrospective study was to compare findings between 24 pediatric (cohort P) and 21 adult HTx patients (cohort A) using optical coherence tomography (OCT) at corresponding post-transplant intervals (≤5 years: P1 (n = 11) and A1 (n = 10); >5 and ≤ 10 years: P2 (n = 13) and A2 (n = 11),. Coronary intima thickness (IT), media thickness (MT) and intima to media ratio (I/M) were assessed per quadrant. Maximal IT >0.3 mm was considered absolute, I/M > 1 relative intimal hyperplasia. RESULTS: Compared to A1, I/M was significantly higher in P1 (maximal I/M: P1: 5.41 [2.81-13.39] vs. A1: 2.30 [1.55-3.62], p = 0.005), whereas absolute IT values were comparable. In contrast, I/M was comparable between P2 and A2, but absolute IT were significantly higher in A2 (maximal IT: P2: 0.16 mm [0.11-0.25] vs. A2: 0.40 mm [0.30-0.71], p < 0.001). A2 presented with higher absolute IT (maximal: A1: 0.16 mm [0.12-0.44] vs. A2: 0.40 mm [0.30-0.71], p = 0.02) and I/M (maximal I/M A1: 2.30 [1.55-3.62] vs. A2: 3.79 [3.01-5.62], p = 0.04). CONCLUSION: Our results suggest an age- and time-dependent difference in the prevalence of absolute and relative intimal hyperplasia in OCT, with an early peak in children and a progressive increase in adults.

Preclinical evaluation of a new self-expanding device for closure of muscular ventricular septal defects in a pig model.

OBJECTIVES: Aim of our study was the preclinical evaluation of a new self expanding device for interventional closure of muscular ventricular septal defects (mVSDs) in an acute pig model. BACKGROUND: Devices currently in use for closure of mVSDs still have their limitations. The deployment of the disks is dependent from the expansion of the stent, which can be associated with problems for sufficient closure of the mVSDs. This was the reason for developing a modified device with only one disk MATERIALS AND METHODS: The device was constructed in a single wire technique with a unique configured retention disk. mVSDs were created in six pigs with a specially designed punch instrument, and subsequently closed with our new device during the same session using a jugular or femoral vein approach. Potential residual shunting volumes were estimated by echocardiography and hemodynamic measurements. After closure, animals were sacrificed, and hearts were harvested for macropathologic evaluation. In two animals, MRI was performed for additional noninvasive evaluation. RESULTS: Devices were successfully implanted in all animals with good alignment of the disk to the left ventricular septum, even if the stent was oversized. Echocardiography, hemodynamics, angiography and macropathology revealed complete closure of all mVSDs. MRI and echocardiography showed a good visibility of the device. CONCLUSIONS: Our preclinical study shows successful closure of iatrogenic created mVSDs without residual shunting. The device is characterized by a more controlled deployment, an independent deployment of disk and waist, and a good alignment of the left ventricular disk to the muscular septum.

Modification of a hybrid technique for closure of muscular ventricular septal defects in a pig model.

OBJECTIVE: Closure of muscular ventricular septal defects (mVSDs) beyond the moderator band is still a challenge for both surgeons and interventional cardiologists. We evaluated a new technique in a pig model for hybrid patch closure of mVSDs via 2 stab wound incisions in the left ventricle (LV) without cardiopulmonary bypass. METHODS: Ten pigs underwent left anterolateral thoracotomy to expose the LV. mVSDs were created via a stab wound incision of the lateral wall of the LV under epicardial echocardiographic control. The patch system was forwarded through a second puncture of the LV apex and positioned in front of the mVSD. The stapler for fixation of the patch was introduced through the same incision as used for VSD creation. Finally, the patch was attached to the septum with nitinol anchors under epicardial echocardiographic and fluoroscopic guidance. Finally, detailed echocardiographic evaluation was done. All hearts were explanted, and macroscopic evaluation was done, either immediately after patch implantation (n = 4) or after 90 days (n = 6). RESULTS: mVSD creation was successful in all pigs. Closure of mVSDs was successful in 8 of 10 pigs, as confirmed by echocardiography, hemodynamic measurements, and macroscopic examination. One patch embolized through the mVSD into the pulmonary artery because of insecure fixation, and 1 animal died during the procedure because of ventricular fibrillation. The final echocardiographic evaluation revealed good LV function and no damage to the valves. CONCLUSIONS: Closure of mVSDs can be successfully performed in a hybrid technique on the beating heart with 2 stab wound incisions; however, further modifications need to be developed before clinical application.

Takotsubo Cardiomyopathy Complicating Percutaneous Pulmonary Valve Implantation in a Child.

Takotsubo cardiomyopathy describes a sudden onset cardiomyopathy with acute impairment of left ventricular function and spontaneous resolution over time. Only a few cases of Takotsubo cardiomyopathy in childhood have been described. We report the case of a 12-year-old girl with repaired tetralogy of Fallot who experienced acute onset of left ventricular dysfunction without coronary arterial involvement, suggesting Takotsubo cardiomyopathy, during an interventional catheterization procedure. Cardiogenic shock necessitated mechanical circulatory support with extracorporeal membrane oxygenator for ten days and mechanical ventilation for 12 days. The girl recovered without sequelae. Percutaneous pulmonary valve implantation was performed four months later without complications. Unusual aspects of this case include the use of mechanical circulatory support during the recovery phase of Takotsubo cardiomyopathy in a patient with congenital heart disease.

Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

OBJECTIVES: The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS: A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3-9.8) years and median weight 12.7 (range 7.6-26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS: Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS: Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.