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. 2022 Feb 16;6(2):ytac067. doi: 10.1093/ehjcr/ytac067
1991 (age 0 years) Male patient born with: Tricuspid atresia with hypoplastic right ventricle, pulmonary stenosis, persisting left superior vena cava, ventricular and atrial septal defect;
Operation (sternotomy # 1): Modified Blalock-Taussig shunt (GORE-TEX® conduit between the brachiocephalic artery and the right pulmonary artery).
1994 (age 3 years) Operation (sternotomy # 2): Bilateral bidirectional cavopulmonary shunts, and patch augmentation of the pulmonary artery confluence.
2002 (age 11 years) Operation (sternotomy # 3): Fontan completion with atriopulmonary connection. Atriopulmonary Fontan with connection of the right atrial appendage to the left pulmonary artery, and atrial septal defect closure using a fenestrated patch.
2006 (age 15 years) Complication: Protein losing enteropathy (PLE) with hypoalbuminaemia, managed with low saturated fat and high protein diet.
2007 (age 16 years) Complication: Pulmonary embolism requiring initiation of oral anticoagulation;
Complication: Recurrent atrial flutter with rapid atrioventricular conduction and sinus node dysfunction, pharmacological treatment (beta-blocker);
Complication: (recurrent) Episodes of cardiac decompensation.
2008 (age 17 years) Angiographic diagnostic procedure: Heart catheterization: low pulmonary artery pressures, obstruction of the pulmonary artery confluence; the right superior vena cava drains into the right pulmonary artery, and the left superior vena cava drains into the left pulmonary artery;
Operation (sternotomy # 4): Conversion to extracardiac total cavopulmonary connection: 20 mm diameter GORE-TEX® tube from inferior vena cava to the left pulmonary artery; resection of atrial septum with reduction of right atrium. Concomitant radiofrequency-ablation (left and right sided), and epicardial DDD-pacemaker implantation (abdominal pulse generator);
Complication: Postoperative PLE and large volumes of pleural effusion leading to re-operation (sternotomy # 5) for creation of a fenestration in the Fontan tunnel (6 mm).
2011 (age 20 years) Complication: Tendency for PLE with hypoalbuminaemia, managed with low saturated fat and high protein diet.
2014 (age 23 years) Complication: Peripheral oedema and fatigue, with desaturation during exercise;
Percutaneous diagnostic procedure: Left-/right heart catheterization: veno-venous collaterals, borderline elevated pulmonary pressures. Pharmacological treatment with phosphodiesterase type 5 inhibitor.
2015–2016 (age 24–25 years) Complication: Recurrent admissions for right-sided decompensation, phosphodiesterase type 5 inhibitor treatment discontinued;
Complication: Hypomagnesaemia (chronic), treated with suppletion therapy.
2015 (age 24 years) Complications: Diagnosis of liver cirrhosis [Child-Pugh A and model for end-stage liver disease (MELD) 6];
Complication: Recurrent concerns about future perspectives and depressive thoughts.
2016 (age 24 years) Invasive diagnostic procedure: Gastroscopy: oesophageal varices grade I-II.
2018 (age 27 years) Complications: Progressive fatigue and desaturation during exercise; attributed to shunting through the veno-venous collaterals.
Complication: Abdominal computed tomography (CT): two foci (10 mm): suspicion for hepatocellular carcinoma (HCC), no metastases.
2019 (age 28 years) Percutaneous intervention # 1: Coiling of veno-venous collaterals (between vena cava inferior and the pulmonary veins).
2019 (age 28 years) Percutaneous intervention # 2: Transarterial chemoembolization of two suspected early stage HCC lesions.
2019–2020 (age 28–29 years) Complication: Non-sustained ventricular tachycardias and short self-terminating paroxysms of atrial tachycardias/flutter: pharmacological treatment (beta-blocker), close follow-up.
2020–2021 (age 29–30 years) HCC diagnosis revised and discarded based on clinical course and revision of CT-scans: better compatible with focal nodular hyperplasia; close follow-up.