Late complications of an early disease

Case presentation: A 62 year-old man with a history of surgically corrected tetralogy of Fallot during late infancy was transported to our hospital for ventricular tachycardia (VT) related syncope. His relevant medical history consisted of an episode of medically managed Streptococcus goordonii-associated infective endocarditis of the pulmonary bioprothesis in 2011, complicated by worsening pulmonary stenosis, requiring the percutaneous implantation of a Melody transcatheter pulmonary valve in 2012. This was followed two years later, in 2013, by another episode of infective endocarditis, with a distinct germ – Streptococcus gallolyticus – of the Melody pulmonary valve, with an uncompli-cated course under prolonged medical therapy. He also had a history of multiple ablation procedures for atrial fibrillation and atypical atrial flutter. Clinical examination did not reveal any obvious anomalies of the cardi-opulmonary system, nor did it reveal signs suggestive of drug abuse in the patient. An ECG tracing during his VT was recorded, demonstrating a left bundle bran-ch morphology of the QRS complexes, with an inferior oriented axis and late transition in the precordial leads, highly suggestive of a VT originating from the RVOT. In contrast, a highly fragmented and large right bundle branch with atrial fibrillation marks his baseline ECG. Lab exams were in normal range, including a comple-te electrolyte panel, TSH, BNP and hs troponine T. He underwent further diagnostic testing in search of an etiology or contributing factor. Coronary angiography revealed no significant coronary artery disease. Becau-se of a very poor acoustic window, transthoracic echo-cardiography was mostly non-contributive, so we deci-ded to perform a gadolinium enhanced cardiac MRI. The latter demonstrated severe RV systolic dysfunction with a RVEF of 25% – a stable finding as compared to his most recent MRI scan performed about a year earlier-, and moderate LV systolic dysfunction with a LVEF of 36% – worsened as compared to his most recent MRI-determined LVEF of 46%. Late gadolinium enhance-ment at the level of the basal and middle segments of the inferior septum was observed. No pulmonary re-gurgitation was noticed. Because of recurring VT des-pite maximally tolerated beta-blockers and amiodaro-ne, the patient underwent successful radiofrequency endocardial ablation of 3 morphologically distinct VTs, two reentrant circuits located in the RV and a focal VT located in the LVOT, with VT non-inducibility at the end of the procedure. No recurrent VT was detected following the ablation on continuous ECG monitoring. Conclusions: Two clinically important questions re-mained: (1) should the patient receive an implantable cardioverter defibrillator (ICD) in secondary preventi-on of sudden cardiac death? (2) if yes, view his history of recurrent infective endocarditis, would a classical endocavitary/epicardial/subcutaneous ICD be prefer-red? The Heart Team, made up of clinical cardiac elec-trophysiology and adult congenital heart disease speci-alists, decided it was best to recommend the patient the implantation of an endocavitary ICD. He underwent successful implantation of a St. Jude MRI compatible single chamber ICD, without any periprocedural com-plication. He was discharged home on optimal medical therapy for heart failure. At 1-month follow up, he was doing well, resumed his favorite activities – playing bo-ules, with no VT recurrence.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)