Andreas Hoschtitzky

Pulmonary Valve Replacement Through a Left Thoracotomy Approach

We report here the first case, to our knowledge, of pulmonary valve replacement being performed via a left thoracotomy approach in a patient with pectus excavatum who had 3 previous sternotomies. The merit of this approach and its feasibility are discussed.

Single ventricle strategy for Uhl's anomaly of the right ventricle

Uhls anomaly is a rare condition and surgical techniques are few and have had variable success. We present a novel and successful surgical technique to treat this difficult condition.

Urgent resection of a giant left atrial appendage aneurysm and mitral valve replacement in a complex case of Hurler-Scheie syndrome.

Hurler-Scheie syndrome is a rare lysosomal storage disease affecting the cardiovascular system. Besides the cardiac manifestations, it presents with complications from abnormal proteoglycan deposition in soft tissues in many locations, resulting in joint contractures, paraplegia, impaired vision, airway narrowing and restrictive lung function, to name a few. There are very few reports of surgical management of valvular heart disease due to mucopolysaccharidosis (MPS). We describe the successful management of a patient with an extremely challenging case of mitral valve stenosis and a giant left atrial appendage aneurysm due to MPS type 1 (Hurler-Scheie syndrome). The patient underwent mitral valve replacement and excision of the giant left atrial appendage aneurysm; a similar case has not been previously reported.

Benefit of endovascular stenting for aortic coarctation on systemic hypertension in adults.

BACKGROUND Endovascular stenting is a recognised treatment strategy for aortic coarctation (CoA) in adults. We assessed systemic hypertension control and the need for antihypertensive therapy after CoA stenting in adults. METHODS Data were collected prospectively on 54 patients (36 men; mean age: 34 ± 16 years) who underwent endovascular stenting for CoA over a 7-year period. Five patients were excluded as they did not attend follow-up appointments. Patients underwent clinical examination, including right arm systolic blood pressure (SBP) and 24-hour ambulatory blood pressure monitoring at baseline, 6-12 weeks and 9-12 months. RESULTS There was a significant fall in mean peak-to-peak systolic gradient (PG) across the CoA after stenting (26 ± 11 mmHg vs. 5 ± 4 mmHg; P<0.01). There were successive reductions in right arm SBP and ambulatory SBP at baseline, 6-12 weeks and 9-12 months post-procedure (right arm: 155 ± 18 mmHg vs. 137 ± 17 mmHg vs. 142 ± 16 mmHg, respectively; all P-values <0.01; ambulatory: 142 ± 14 mmHg vs. 132 ± 16 mmHg vs. 131 ± 15 mmHg, respectively; all P-values <0.01). Twenty-four patients had severe CoA (PG >25 mmHg before stenting); baseline SBP was significantly higher in severe versus non-severe patients (160 mmHg vs. 148 mmHg; P=0.02). The absolute reduction in PG after stenting was significantly higher in the severe group (31 ± 7 mmHg vs. 14 ± 5 mmHg; P<0.0001), but there was no significant difference in SBP between groups at 6-12 weeks (141 mmHg vs. 135 mmHg; P=0.21) or 9-12 months (139 mmHg vs. 139 mmHg; P=0.96). CONCLUSION Endovascular stenting of CoA results in a significant reduction in SBP at 6-12 weeks, which is sustained at 9-12 months, with similar outcomes in severe and non-severe CoA groups.

Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly

BACKGROUND Accurate risk stratification of patients with Ebsteins anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ± 7.7% vs 36.7 ± 9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ± 6.2% vs -16.2 ± 7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ± 13.5% vs 36.3 ± 12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

P27 An evaluation of patient outcomes following percutaneous pulmonary valve implantation from a single centre

Background Congenital right ventricular outflow tract (RVOT) dysfunction can now be treated percutaneously. There is demonstrable evidence that percutaneous pulmonary valve implantation PPVI effectively restores conduit graft viability with a consequent improvement in right ventricular pressures and exercise tolerance. Aim This study evaluates early and late peri-procedural factors in patients with RVOT dysfunction, following PPVI using Medtronic Melody (MM) and Edwards SAPIEN (ES) valves. Methodology This was a single-centre retrospective study of a cohort of 41 consecutive men (n = 18) and women (n = 23), mean age 26.1 ± 10.2 (±SD) years who underwent PPVI between December 2007 and November 2014. Indications for PPVI were significant homograft dysfunction in the context of previously corrected pulmonary atresia (n = 10), pulmonary stenosis (PS) or regurgitation (Ross procedure, n = 9), Transposition of Great arteries (n = 4), Tetralogy of Fallot (n = 15), and truncus arteriosus (n = 3). Patients were categorised as having pulmonary stenosis (44%), regurgitation (34%) or mixed pulmonary valve disease (22%). Invasive haemodynamic, echocardiographic, electrocardiographic (ECG) and cardiopulmonary exercise test (CPET) parameters were assessed pre and post-procedure, at 1, 6 and 12 months (and at 2 or 3 years respectively in some patients). Differences between the measurements were tested by ANOVA or with χ2 test for proportions. Abstract P27 Figure 1 Trends in maximal main pulmonary artery pressure gradient over time. Results PPVI significantly reduced the mean baseline RVOT gradients in stenosis (45 vs18.4 mmHg); regurgitation (19.2 vs. 7.6 mmHg) and mixed disease (32.5 vs. 12mmHg). Mean right ventricular (RV) systolic pressures fell (61.6 ± 2.3 vs. 41.9 ± 2mmHg, p < 0.001) and RV diastolic pressures decreased by about 60% (14.3 ± 1.1 vs. 8.6 ± 1.4mmHg, p < 0.001). Echocardiography showed a clear improvement in pulmonary and tricuspid valve velocities (p for trend <0.01). Furthermore, there was consistent reduction in the main pulmonary artery maximum pressure gradient measured pre- procedure (Figure 1). No significant changes in duration of PR, QRS or QTc were apparent on follow-up ECGs. No changes were observed in CPET performance during follow-up. Discussion Implantation of MM and ES valves in patients with pulmonary stenosis, regurgitation or mixed pulmonary diseases appear to be effective. Immediate improvement in right ventricular pressures and functional improvements demonstrated by echocardiography suggest PPVI is a worthwhile treatment option for patients with dysfunctional RVOT homografts. Multi-centre collaborations are essential to further determine long-term effects of PPVI on cardiac function, exercise tolerance and quality of life in RVOT dysfunction.

160 Arrhythmia and Heart Failure Substrates in the Right Ventricular Outflow Tract of Adults with Surgically Repaired Tetralogy of Fallot

Introduction Adults with Tetralogy of Fallot (ToF) have an increased risk of heart failure, arrhythmias and sudden death. We sought to investigate whether myocardial remodelling of the right ventricular outflow tract (RVOT) could be responsible. Methods We collected RVOT myocardial biopsies from 10 ToF patients (6 male) at the time of pulmonary valve replacement. As controls, biopsies from 9 patients (4 male) with left ventricular outflow tract obstruction without evidence of arrhythmia or right-sided heart disease on clinical examination, ECG and echocardiography were collected. We performed: (i) picrosirius red to quantify extracellular matrix (ECM); (ii) qPCR to quantify mRNA for ion channels, transporters, connexins, inflammatory markers and constituents of the ECM; (iii) proteomics (Liquid Chromatography and Mass Spectrometry,MS; on 6 patients per group) to quantify ~2000 proteins; and (iii) Western Blot to quantify selected proteins. Results Mean age during surgery was 32 ± 4 and 22 ± 2 years in the ToF and control groups, respectively. There was no significant difference in the amount of fibrosis on histological analysis. In the ToF patients, there were significant changes in the relative abundance of 19 mRNAs for ion channels, adrenergic receptors, ECM, and two heart failure markers (ANP and BNP). MS highlighted over 300 significant changes between ToF and control patients. A selection of changes identified on qPCR and MS are displayed in Table 1. Abstract 160 Table 1 Conclusions The RVOT undergoes significant remodelling in ToF. Changes in ion channels and transporters in ToF patients might alter ionic currents. Risk of arrhythmia may be increased by changes to Ca2+-handling and gap junction protein, Cx43. Changes are similar to those described in heart failure and are possibly contributing to the high arrhythmia burden seen in TOF.