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A Case of Noncompaction Cardiomyopathy
Cardiovascular Disease SESSION TITLE: Student/Resident Case Report Poster - Cardiovascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
A Case of Noncompaction Cardiomyopathy Behnaz Parsamehr MD* Marcelle Meseeha MD; and Victor Kolade MD Guthrie-Robert Packer Hospital, Sayre, PA
CARDIOVASCULAR DISEASE
INTRODUCTION: Left Ventricular Noncompaction Cardiomyopathy (LVNC) is a rare form of cardiomyopathy with an abnormal thick myocardium and multiple trabeculations of endocardial layer. Clinical manifestation of LVNC includes chest pain, syncope, arrhythmia, sudden cardiac arrest, and thromboembolism. Electrocardiogram in these patients could show different abnormalities such as left or right bundle branch block, fascicular block, atrial fibrillation, and ventricular tachycardia. An association of LVNC with sinus bradycardia or Wolff-Parkinson-White syndrome has been described in some cases [1]. CASE PRESENTATION: A 20 year old African American male had multiple episodes of palpitation and chest pain. Patient did not have any significant past medical history other than hypercholesterolemia. Patient did not have history of smoking, alcohol, or drug abuse. Two years ago he woke up with an episode of palpitations and chest pain that prompted an emergency room visit. He was found to have T wave inversions in pre-cordial leads. A subsequent echocardiogram revealed extensive trabeculations in the apex of the LV with LVEF of 35%. A cardiac MRI was performed which showed increased ratio of noncompacted wall to compacted wall (2.5:1) in addition to global hypokinesis of the left ventricle. Patient started taking Nebivolol and later he switched to metoprolol. Patient also started taking Lisinopril and warfarin for his cardiomyopathy. One year later, he came to the ER with an episode of prolonged palpitation. He was found to have an episode of nonsustained ventricular tachycardia and subsequently a subcutaneous defibrillator was placed for him. Two months later he developed Atrial Fibrillation (AF) with rapid ventricular response (RVR). Patient was medically managed with Cardizem infusion. Interrogation of ICD revealed AF with heart rate in 200s. He started taking Sotalol and the next day he converted to sinus rhythm. DISCUSSION: Noncompaction cardiomyopathy is a rare type of cardiomyopathy with different manifestations such as heart block, atrial, and ventricular arrhythmia. Genes have been recognized to play a key role in pathogenesis of this condition. Screening of up to 3 generations of affected individuals has been recommended. High index of suspicion is necessary in order to diagnose LVNC. CONCLUSIONS: There are common genes involved in pathogenesis of hypertrophic cardiomyopathy and LVNC. Most of the times LVNC is misdiagnosed as hypertrophic cardiomyopathy. Our patient was diagnosed with a rare type of cardiomyopathy at an early stage. Placement of AICD was a good decision for this patient. What we concluded from this case was that a high suspicion for certain disorders in addition to proper diagnostic test is the key in diagnosing and managing the patients. Reference #1: Attenhofer Jost, Christine H., and Heidi M. Connolly. “Left ventricular noncompaction: dreaming of the perfect diagnostic tool.” European journal of heart failure 14, no. 2 (2012): 113-114. DISCLOSURE: The following authors have nothing to disclose: Behnaz Parsamehr, Marcelle Meseeha, Victor Kolade No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.098
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
90A
[
150#4S CHEST OCTOBER 2016
]
A Case of Noncompaction Cardiomyopathy Behnaz Parsamehr MD* Marcelle Meseeha MD; and Victor Kolade MD Guthrie-Robert Packer Hospital, Sayre, PA
CARDIOVASCULAR DISEASE
INTRODUCTION: Left Ventricular Noncompaction Cardiomyopathy (LVNC) is a rare form of cardiomyopathy with an abnormal thick myocardium and multiple trabeculations of endocardial layer. Clinical manifestation of LVNC includes chest pain, syncope, arrhythmia, sudden cardiac arrest, and thromboembolism. Electrocardiogram in these patients could show different abnormalities such as left or right bundle branch block, fascicular block, atrial fibrillation, and ventricular tachycardia. An association of LVNC with sinus bradycardia or Wolff-Parkinson-White syndrome has been described in some cases [1]. CASE PRESENTATION: A 20 year old African American male had multiple episodes of palpitation and chest pain. Patient did not have any significant past medical history other than hypercholesterolemia. Patient did not have history of smoking, alcohol, or drug abuse. Two years ago he woke up with an episode of palpitations and chest pain that prompted an emergency room visit. He was found to have T wave inversions in pre-cordial leads. A subsequent echocardiogram revealed extensive trabeculations in the apex of the LV with LVEF of 35%. A cardiac MRI was performed which showed increased ratio of noncompacted wall to compacted wall (2.5:1) in addition to global hypokinesis of the left ventricle. Patient started taking Nebivolol and later he switched to metoprolol. Patient also started taking Lisinopril and warfarin for his cardiomyopathy. One year later, he came to the ER with an episode of prolonged palpitation. He was found to have an episode of nonsustained ventricular tachycardia and subsequently a subcutaneous defibrillator was placed for him. Two months later he developed Atrial Fibrillation (AF) with rapid ventricular response (RVR). Patient was medically managed with Cardizem infusion. Interrogation of ICD revealed AF with heart rate in 200s. He started taking Sotalol and the next day he converted to sinus rhythm. DISCUSSION: Noncompaction cardiomyopathy is a rare type of cardiomyopathy with different manifestations such as heart block, atrial, and ventricular arrhythmia. Genes have been recognized to play a key role in pathogenesis of this condition. Screening of up to 3 generations of affected individuals has been recommended. High index of suspicion is necessary in order to diagnose LVNC. CONCLUSIONS: There are common genes involved in pathogenesis of hypertrophic cardiomyopathy and LVNC. Most of the times LVNC is misdiagnosed as hypertrophic cardiomyopathy. Our patient was diagnosed with a rare type of cardiomyopathy at an early stage. Placement of AICD was a good decision for this patient. What we concluded from this case was that a high suspicion for certain disorders in addition to proper diagnostic test is the key in diagnosing and managing the patients. Reference #1: Attenhofer Jost, Christine H., and Heidi M. Connolly. “Left ventricular noncompaction: dreaming of the perfect diagnostic tool.” European journal of heart failure 14, no. 2 (2012): 113-114. DISCLOSURE: The following authors have nothing to disclose: Behnaz Parsamehr, Marcelle Meseeha, Victor Kolade No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.098
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
90A
[
150#4S CHEST OCTOBER 2016
]