Prevented Sudden Cardiac Death and Neurologic Recovery in Inherited Heart Diseases

Abstract

Introduction: Inherited cardiovascular diseases are an important cause of sudden cardiac death (SD). The use of risk scores identify high risk patients who would benefit from an implantable cardioverter-defibrillators (ICDs). The development of automated devices for out-of-hospital cardiac arrest improves early resuscitation. The objective of the study is to quantify prevented SD and the neurological recovery of patients with inherited cardiovascular diseases. Methods: Two hundred fifty-seven cases of SD (age 42 ± 18 years, 79.4% men) of non-ischemic cardiac cause were prospectively collected during the study period (2009-17). Fifty three (20.6%) had a resuscitated cardiac arrest (RCA) (age 40 ± 18 years, 64.2% male). Epidemiological, clinical and autopsy aspects were analyzed. Prevented SD was defined as a combination of RCA and appropriate ICD therapy cases. Results: An autopsy was performed in 157/204 (77.0%) cases who died. There were 19 (12.1%) cases with a negative autopsy. The diagnosis of cardiomyopathy and channelopathy was 58.0 and 18.7%, respectively. Female sex and confirmed or suspected channelopathy were associated with successful resuscitation. The percentage of prevented SD remained low during the study period (mean 35.6%). 60.4% of RCA cases presented good neurological outcome. There was no association between neurological recovery and therapeutic hypothermia, but there was association with time of resuscitation (min). Conclusion: A fifth part of non-ischemic cardiac arrests were resuscitated. Female sex and channelopathies were more prevalent among RCA. Two thirds of RCA had a good neurological recovery.

Keywords: cardiomyopathy; channelopathy; inherited cardiovascular diseases; prevented sudden cardiac death; sudden cardiac death.

Figure 1

Study design. Selection of population and objectives. ILM, Institute for Legal Medicine and Forensic Sciences; ICDU, Inherted Cardiac Disease Unit; SD, Sudden death; RCA, Resuscitated Cardiac Arrest.

Figure 2

Final diagnoses of cases of resuscitated and non-resuscitated SD included in the study (autopsy or clinical study). Hypertrophic cardiomyopathy (HCM): includes 80 cases with HCM and 17 with idiopathic left ventricular hypertrophy. Arrhythmogenic cardiomyopathy (ACM): includes 24 cases with right ventricle and 11 with left ventricle involvement.

Figure 3

Histology of the different causes of sudden death. (A) and (B): respectively, macro and microscopy (with hematoxylin and eosin stain) of a morphologically normal heart. (A) 3-mm-thick right ventricular wall, 12-mm-thick left ventricular wall; (B) bundles of striated muscle can be seen perfectly in line with sarcoplasms with their normal transverse striations and normochromatic oval nuclei (14-year-old female who died during an argument, diagnosed with catecholaminergic polymorphic ventricular tachycardia). (C) and (D) respectively, macro and microscopy (with Masson's trichrome stain) of hypertrophic cardiomyopathy. Fibrosis and disarray can be seen (40-year-old male who died at work). (E) Macroscopic appearance of dilated cardiomyopathy. The rounding appearance of the apex stands out. (F) Masson's trichrome stain of a heart with dilated cardiomyopathy. Patchy interstitial fibrosis surrounding muscle fibers (45-year-old male who died at home). (G) Section of a heart with large fatty infiltration, particularly at the right ventricle, in an arrhythmogenic cardiomyopathy. (H) microscopically, a massive fatty infiltration can be seen in the right ventricular wall. (I) subtotal fibro-fatty infiltration in the left ventricle, it extends more intensely from the epicardium to the endocardium (39-year-old male who died in his sleep).