While virus epidemics are nothing new to man, the scale, speed of global spread and immediacy of the COVID-19 pandemic have been truly unprecedented [ 1 ]. The entire world has been turned on its head in less than a few months, with major implications beyond disease burden and loss of life, threatening the economic status quo and human psychosocial balance and wellbeing not only for patients, but for all of us. The primary aim of our Call for Action Viewpoint was to support and protect our adult congenital heart disease (ACHD) patients and their needs during these challenging and uncertain times. This goal had to be met while we, as individuals, teams, institutions and nations, came together in a global effort to combat this aggressive virus, that appears to spare no organs or systems, nor any borders, geographic or other. As with any crisis, there is always opportunity: we are submitting herewith a vision for a different and better model of ACHD care, and for a better life journey and health care experience for our patients, that should be in place in the aftermath of the Covid-19 pandemic. Many of the points and principles discussed in this article, need not be confined to ACHD patients, but have a broader reach. And we must not forget nor neglect the most vulnerable in society at this time, namely the elderly, disabled and other dependent or disadvantaged groups in this “We Are One” global operation. Last but not least, this maybe the time to take better care of ourselves (and others) and reflect on life .

We present the challenging case of a young man with congenital heart disease who survived severe device-related infective endocarditis and new pulmonary hypertension. He required prolonged mechanical circulatory support and had multiple significant complications. His case posed a management dilemma that was successfully resolved by effective multidisciplinary, tertiary center care. ( Level of Difficulty: Beginner. )

A middle aged male with history of Fallot type Double outlet right ventricle and coarctation of aorta who underwent multiple surgeries since childhood. With RV to pulmonary artery conduit and end to end coarctation repair initially, followed by aortic valve, pulmonary valve and RV-PA conduit replacements later. He subsequently developed significant flow through a fistula between a previously coiled aortic pseudoaneurysm and LVOT. This was percutaneously closed with a vascular plug and additionally a PFO, detected, was closed with a device. Patient returned to the clinic with decompensated heart failure. Updated imaging demonstrated that the plug had migrated and together with the coil, which was impinging on the AVR, resulting in severe supra-valvar AS and continuous significant persistent residual flow across the Aorta- LVOT fistula via false aneurysm. Our case highlights the importance of using multimodality imaging for complex congenital heart diseases. In view of high surgical risks associated with previous multiple sternotomies and patient’s preference, percutaneous approaches were adopted initially: however, it was unfortunate to see the migration of vascular plug. After successful deployment of AVP, migration or recanalization is rare, but should not be completely discounted.

Background/Introduction Complete Atrio-Ventricular septal defect (AVSD) is a complex congenital cardiac disease, characterised by malformation at the atrio-ventricular junction and AV valve abnormalities. Long- term outcome data is limited. Purpose We sought to describe the long-term outcomes of adults with repaired complete AVSD in a tertiary adult congenital heart centre. Methods We retrospectively recruited patients with complete AVSD who underwent surgical repair between 1973 and 2001 in our centre. All clinical and echocardiographic data were collected and analysed for evidence of atrio-ventricular valve (AVV) deterioration, or cardiac dysfunction. We also assessed for the requirement for further surgical intervention throughout the follow up period. Results A total of 345 patients with AVSD were identified, partial AVSD 211, unrepaired 82. Fifty-two with repaired complete AVSD formed study group, female 36 (69%) and male 16 (31%), Trisomy 21 (23, 44%). Mean age at initial repair was 44 months (median 12, IQR 31.5). Mean follow up was 25.3 years (Median 24, IQR 9.75). Clinical status: At the latest follow-up, most patient were asymptomatic with NYHA I-II (n=46) Majority (n=36, 69%) required only initial repair and no further intervention. Further surgeries were performed in 16 patients including AVV repair (n=9) and LVOTO relief (n=3). Permanent pacemaker insertion needed in 6 (12%), all for heart block following valve repair. 7 patients (14%) had documented arrythmia, 4 (8%) requiring ablations and only 1 had endocarditis. Echo findings: AV Valve dysfunction was more commonly regurgitant rather than stenotic. More than moderate AVV regurgitation was present in 83% of patients, Left AVV (n=25, 48%), Right AVV (n=18, 35%) compared to 4% stenosis (LAVV n=1, RAVV n=1). Ventricular outflow tract obstruction was present in 8% of patients, RVOT obstruction (2, one native, one secondary to prior banding), LVOT obstruction (2, both native, one requiring surgical intervention). Right ventricular systolic dysfunction was present in 6% of patients (n=3), with mean TAPSE 14.1mm (SD± 3.1mm). Left ventricular dysfunction was present in 4% (n=2), mean LVEF 58.9% (SD±7.1%) and mean LV EDVi 55.06mL/m2 (SD±13.2 mL/m2). Only one patient had significant pulmonary hypertension (Mean PAP 48 mmHg). Conclusion Long-term outcomes of surgically repaired AVSD are highly favourable. Left AVV regurgitation is the most common residual lesion requiring further surgical intervention. Ventricular outflow tract obstruction was much less common, as was ventricular systolic dysfunction. Complete heart block was associated with surgical repair and arrhythmias were potential late complications. FUNDunding Acknowledgement Type of funding sources: None.