6/12/2017. Isolated Noncompaction of the Left Ventricle. Objectives. Pathophysiology

Transcription

1 Isolated Noncompaction of the Left Ventricle Beth Davidson DNP, ACNP, CHFN, CCRN AAHFN June 23, 2017 Objectives 1. Describe the pathophysiology of LVNC 2. Identify the key clinical findings/manifestations of LVNC 3. Discuss management strategies for LVNC No disclosures related to this presentation Pathophysiology Genetic vs. unclassified cardiomyopathy Characterized by Altered myocardial wall with prominent trabeculae and deep intratrabecular recesses Continuity between LV cavity and recesses without evidence of communication to coronary arteries 1

2 Pathophysiology Isolated condition or seen in association with other abnormalities Congenital right or left ventricular outflow tract disorders Bicuspid aortic valve, hypoplastic left heart syndrome, atrial/ventricular septal defects Neuromuscular disorders/metabolic diseases/genetic syndromes Histological Features A: anastomosing B: coarse C: spongy E: compressed A.Bruke et al, Human Pathology 2005; 36, Epidemiology Difficult to determine Nonstandardized diagnostic criteria Estimated to be 3 4% in heart failure population Likely an underestimate Enhanced recognition with improved imaging modalities 2

3 Genetics Heterogenous familial or sporadic Major gene mutations Taffazin Alpha-dystrobrevin (DTNA) Sarcomeric protein genes Hussein, et al JACC, 66 (5), 2015, Clinical Manifestations Heart failure Atrial and ventricular arrhythmias, conduction abnormalities Thromboembolic events Sudden cardiac arrest Screening/Diagnosis EKG changes nonspecific Diagnostic criteria evolving and requires further standardization 2D echo Cardiac MRI Computed tomography (CT) Explant/Autopsy Differential diagnosis Hypertensive heart disease Other cardiomyopathies dilated, hypertrophic, restrictive 3

4 Echocardiography Jenni criteria Thickened LV wall consisting of 2 layers thin compacted epicardial layer, markedly thickened endocardial layer Color doppler evidence of flow within the deep intratrabecular recesses Prominent trabecular meshwork in the LV apex or midventricular segments 2D Echo 4

5 Cardiac MRI Superior diagnostic modality for visualization of LV noncompaction Management Strategies Varies with clinical manifestations, ejection fraction, presence of arrhythmias, risk of thromboembolism GDMT (HFrEF) Anticoagulation ICD for primary/secondary prevention, pacemaker Family screening/genetic counseling Avoid competitive/endurance sports Heart transplant evaluation Prognosis Associated with high rates of morbidity and mortality in adults arterial thromboembolism tachyarrhythmias/sudden cardiac death/heart block heart failure decompensation Outcomes related to severity at clinical presentation 5

6 Case Study 34 yr. old Hispanic male HF secondary to LV noncompaction Initially diagnosed 2002 with CVA Previously evaluated at VUMC with hyperdynamic EF Atrial fibrillation Warfarin 2D echo 7/2009 EF 45, marked LV trabeculations, moderate MR, severe TR s/p ICD for primary prevention Transplant Evaluation Referred for transplant evaluation Right heart catheterization (10/2010) Normal filling pressures, PAS 50-80, CI Discharged home: status 1B Milrinone mcg/kg/min Revatio 20mg tid Transplantation vs. VAD Readmitted (11/30/2011) HF exacerbation, NYHA IV, failure to thrive Repeat RHC: PAS Diuresis Flolan (IV and inhalation) Nipride Revatio Milrinone 6

7 The Wait Begins Recurrent Afib DCCV 12/7 and 12/17 Non-infectious hepatitis UTI Enterobacter PNA Acute kidney injury Severe protein malnutrition Cardiogenic shock Transplantation PAS 40 50s with TPG < 15 Upgraded to Status 1A Txpl and removal of ICD 12/29/2010 Post-op: Nitric oxide, Flolan, Milrinone ARF secondary to CNI brief dialysis support (L) thoracentesis RHC: RA 5, PA 37/21, WP 14, CI 3.4 Endomyocardial bx: negative for rejection ICD pocket hematoma evacuated 1/7/2011 Home: 1/10/2011 7