INTRODUCTION: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare arrhythmogenic disorder characterized by stress induced ventricular tachyarrhythmia resulting in syncope or sudden death. We present a case of a young girl with CPVT presenting in cardiogenic shock treated with cardiac sympathetic denervation.
CASE PRESENTATION: A 9-year old girl presented after a near drowning even in cardiogenic shock secondary to refractory ventricular tachycardia (VT). Despite multiple attempts at cardioversion as well as lidocaine and high-dose beta-blockers, the patient remained in VT necessitating ECMO support to maintain circulation and hemodynamics. She was then transferred to our institution for further management. Further questioning revealed a history of episodic exercise induced syncope and questionable seizures and inspection of her EKG showed bidirectional ventricular tachycardia characteristic of CPVT. She was taken to the operating room for left cardiac sympathetic denervation consisting of resection of the T1-T4 ganglia via left thoracoscopy. Following this procedure she remained in NSR and was weaned from ECMO two days later. Intravenous administration of a beta-blocker was initially continued while the patient made a satisfactory recovery from surgery. The following hospital course was complicated by cholecystitis eventually requiring cholecystectomy and a prolonged recovery from a neuropsychological perspective. On multiple occasions during this complicated hospital course the patient developed cardiac tachyarrhythmias although she always remained hemodynamically stable and had no further episodes of sustained VT. Yet, in view of the underlying disease and risk of lethal arrhythmias implantation of an ICD as a preventive measure was warranted. She was ultimately discharged home 32 days after her VATS with left cardiac sympathetic denervation. An uneventful further recovery followed at home. During the subsequent 18 months of follow up she remained asymptomatic from a cardiovascular standpoint, had an unrestricted, age-appropriate physical activity level and no recorded events of VT or shocks fired by the ICD.
DISCUSSION: CPVT, first described in 1975, is a rare genetic arrhythmogenic disorder with an estimated prevalence of 1:10,000. It is a disorder of abnormal myocardial calcium homeostasis characterized by life-threatening ventricular arrhythmias triggered during states of high sympathetic output. The mainstay of treatment is protection of the unstable heart both pharmacologically with beta blockers and mechanically with placement of an ICD . Even with these interventions, episodes of VT can occur and can result in the phenomenon of “electrical storms” in which the first ICD shock restores NSR. However, the pain and fear associated with this can cause another surge of catecholamines triggering further arrhythmic episodes followed by ICD shocks resulting in a vicious cycle. In these cases, cardiac sympathetic denervation consisting of division of the left sympathetic chain from T1-T4 has proven useful , and in our case life-saving. This surgery can be done thoracoscopically via 2 or 3 small (1cm) incisions with minimal morbidity .
CONCLUSIONS: CPVT, although rare, should be considered in all young patients presenting with ventricular arrythmias or with a history of stress/exercise induced syncope. Beta blockers and ICD placement are the mainstays of treatment, however, thoracoscopic cardiac sympathetic denervation, a procedure with minimal morbidity, is a valuable surgical adjunct in difficult to control cases or in life-threatening situations.
Reference #1 Sumitomo N, Harada K, Nagashima M, Yasuda T, Nakamura Y, Aragaki Y, Saito A, Kurosaki K, Jouo K, Koujiro M, Konishi S, Matsuoka S, Oono T, Hayakawa S, Miura M, Ushinohama H, Shibata T, Niimura I. Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death. Heart. 2003 Jan;89(1):66-70
Reference #2 Wilde AA, Bhuiyan ZA, Crotti L, Facchini M, De Ferrari GM, Paul T, Ferrandi C, Koolbergen DR, Odero A, Schwartz PJ. Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. N Engl J Med. 2008 May 8;358(19):2024-9.
DISCLOSURE: The following authors have nothing to disclose: Moritz Wyler Von Ballmoos, Ghulam Murtaza, James Tweddell, Mario Gasparri
No Product/Research Disclosure Information