Patients with complete transposition of the great arteries (TGA) treated by the Senning procedure have a higher risk of developing heart failure due to: a) additional work load of the systemic (morphologic right) ventricle (sRV), b) arrhythmias, mainly caused by surgical implications at the atria as well as c) worsening of systemic tricuspid regurgitation. We present a unique case of a female patient who developed all these complications, who was successfully treated and was able to carry out a twin pregnancy. This breakthrough approach was based on: 1. detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia, and 2. prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing. Surgical replacement of systemic tricuspid valve (sTV) and cardiac resynchronization device (CRT) implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV. The reversal of a further decline in systolic function was achieved by permanent arrhythmia control, synchronous pacing with epicardial leads of CRT, sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery. Two years after delivery, the patient remains in NYHA Class I.
Background:Constrictive pericarditis is a rare disease but can cause diagnostic problems. The diagnosis can be even more difficult if combined by pulmonary emobolism 1,2 . We present the case of constrictive pericarditis complicated with several thromboembolic incidents.case Presentation: A 58-year-old was admitted to Clinical Hospital with symptoms of dyspnoea, ortopnoe, fatigue and signs of dominantly right heart congestion. As a 6-year old child he had left sided pleuritis. Later, he was working at shipyard in the region close to asbestos factory. Four years before admission he was accidentally diagnosed atrial fibrillation. Anticoagulant therapy with dabigatran was started. Electrocardioversion was done successfully for three times and finally radiofrequency ablation had been planned when patient felt breathless suddenly. He was admitted to local hospital where thoracic MSCT showed bilateral incapsulated pleural effusion, bronchial deformities and a small pericardial effusion that measured 0.7 cm. Right sided pneumonia was suspected so patient was treated with antibiotics but with no benefit. MSCT pulmonary angiography did not show pulmonary embolism, but verified worsening of pericardial effusion, hepatic congestion and ascites. PET CT showed no pathology. Fiber optic bronchoscopy found only nonspecific mucopurulent substrate. Quantiferon test was negative. Dyspnoea was worsening, as well as pericardial effusion. Anticoagulant therapy had been stopped for a while. Echocardiography examination found dilatation of both atria with an indirect signs of high right atrial pressure and constrictive hemodynamic and thrombus in right atrium (Figure 1). Control CT showed multiple thrombotic masses in right atrium, segmental pulmonary arteries, left atrial auricular. Doppler did not show peripheral vein thrombosis. Anticoagulant therapy was started again. Searching for thrombophilia found normal FV, FII, MTHFR (CC gene type), polymorphism (4G/5G) for PAI-I, and insertion ACE genotype. Polyserositis was suspected so corticosteroid wad added to therapy. Heart MR confirmed constrictive pericarditis and made suspicious of thrombotic masses in both atria. Meanwhile patient had another thromboembolic episode; inferior and superior caval vein thrombosis, hepatic and renal veins thrombosis, and right iliac artery embolism. Iliac artery trombendarterektomia was successfully done. Patient was transferred to the tertiary hospital for the diagnostic confirmation of constrictive pericarditis.
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