Cardiology Letters 2020, 29(4):259-267

Supraventricular tachycardia in idiopathic pulmonary arterial hypertension

Luknár M¹, Lesný P¹, Svetlošák M², Hlivák P², Goncalvesová E¹

¹ Kardiologická klinika Lekárská fakulta a Národný ústav srdcových a cievnych chorôb, a. s., Bratislava, Centrum pre pľúcnu artériovú hypertenziu

² Oddelenia arytmií a kardiostimulácie, Národný ústav srdcových a cievnych chorôb, a. s., Bratislava, Slovenská republika

Supraventricular arrhythmias in pulmonary arterial hypertension (PAH) are common and often result in severe clinical and hemodynamic deterioration. The management is complex and involves conservative and interventional approaches. We present a case of a patient with idiopathic PAH with multiple recurrent supraventricular arrhythmias. The female patient (b. 1978) was diagnosed with idiopathic PAH in 2006 and sildenafil treatment was initiated in 2007. Due to disease progression, SQ treprostinil was started in 2012 and bosentan was added in 2013. In April, 2014, she was admitted to hospital due to abrupt worsening of dyspnea and hypotension. Regular tachycardia with the rate of 150/min was detected and terminated by intravenous adenosine. On electrophysiology study (EPS), atrio-ventricular nodal re-entrant tachycardia (AVNRT) was confirmed and a successful slow pathway modulation by radio frequency (RF) ablation was performed. In addition, several regular and irregular atrial tachycardias other than typical atrial flutter were inducible by isoprenaline at EPS. A small dose of short-acting beta-blocker (metoprolol) was initiated and the patient was discharged from hospital in a clinically stable status and in sinus rhythm. However, she was re-admitted to hospital due to dyspnea at rest, signs of right ventricular failure, and persisting atrial tachycardia 6 weeks later. Electric cardioversion was performed and sinus rhythm was restored. Amiodarone was added. Further interventional treatment was not indicated due to high risk and low probability of success of the procedure in context of multiple foci of arrhythmia. Atrial tachycardia recurred soon and persisted. Unfortunately, the patient´s status further deteriorated in spite of treatment escalation and she was listed for lung transplant. Atrial tachycardia with adequate ventricular rate persisted in the long-term. The patient died of heart failure early after lung transplant.

Conclusion: SV arrhythmia can result in an abrupt destabilization of the patient´s status. We believe it is of the utmost importance to aim for reconstitution of the sinus rhythm using all available conservative and interventional approaches. On the other side, it is also possible that SV arrhythmia represents one of the signals of disease progression and a marker of unfavourable outcome. We therefore presume that on the occurrence of SV arrhythmia, pulmonary hypertension specific treatment escalation should be considered to minimize further PAH progression. Fig. 4, Tab. 3, Ref. 18, on-line full text (Free, PDF) www. cardiologyletters.sk

Keywords: supraventricular arrhythmias; idiopathic pulmonary arterial hypertension; tachycardia; dyspnea

Published: April 1, 2020  Show citation