Table 1.
Timeline of the case
| Date | Relevant medical history and interventions | ||
|---|---|---|---|
| Summaries from initial and follow-up visits | Diagnostic testing | Interventions | |
| November 25, 2016 | A 55-year-old African American woman with a history of hypertension and smoking 1–2 cigarettes per d for 10 years presented with progressive shortness of breath, cough with copious pink frothy sputum, and chest pain that started on the day of presentation. Initial diagnostic assessment was acute respiratory failure because of acute pulmonary edema and new-onset congestive heart failure. History also included 1–2 glasses of wine twice a week and paternal diabetes mellitus. |
Laboratory and imaging studies (Nov 25): NT pro-BNP, 5911 pg/mL; troponin, 0.022 ng/mL that peaked at 2.360 ng/mL ECG: New LBBB; QTc, 442 milliseconds CT of chest: Bilateral pulmonary edema, right-sided aortic arch Transthoracic echocardiography (Nov 28): LVEF, 30%–35%; moderate to severe aortic regurgitation, severe mitral regurgitation Transesophageal echocardiography (Nov 29): LVEF, 25%–30%; severe aortic regurgitation, moderate mitral regurgitation, endocardial wall highly suggestive of left ventricular noncompaction Cardiac catheterization (Nov 30): No substantial coronary artery disease |
Patient was emergently intubated and started on intravenous diuresis. Guideline-directed medical therapy for heart failure (β-blocker and ACE inhibitor) was gradually introduced once acute pulmonary edema resolved. She was subsequently extubated. A low-salt, low-fat diet was started. Smoking cessation counseling was initiated. A wearable defibrillator was provided before her discharge on December 2, 2016. |
| December 23, 2016 | Outpatient tests | Cardiac MRI: Showed left ventricular noncompaction | |
| December 25, 2016 | Patient presented with syncope and subsequent shock from her wearable defibrillator. | Wearable defibrillator interrogation (Dec 25): Polymorphic ventricular tachycardia | Serum electrolyte levels were corrected. Wearable defibrillator was continued in anticipation of CRT-D implantation if LVEF remained low after 3 months of optimal medical therapy for heart failure. Anticoagulation therapy was started because of increased risk of thromboembolism with noncompaction diagnosed on cardiac MRI. Patient was discharged home on December 27, 2016. |
| January 30, 2017 | Patient presented for aortic valve replacement and mitral valve repair. She had a prolonged and complicated hospital course because of acute respiratory failure requiring mechanical ventilator support. | Aortic valve replacement and mitral valve repair (February 20). Mitral valve replacement (March 10). Cardiac rehabilitation was initiated, and her clinical status improved. | |
| March 26, 2017 | Sudden cardiac arrest developed because of ventricular fibrillation, and she could not be resuscitated. | ||
ACE = angiotensin-converting enzyme; CRT-D = cardiac resynchronization therapy with defibrillator; CT = computed tomography; ECG = electrocardiography; LBBB = left bundle branch block; LVEF = left ventricular ejection fraction; MRI = magnetic resonance imaging; NT pro-BNP = N-terminal pro-brain-type natriuretic peptide.