Table 1.
The characteristics of the patients presented in the current case series.
| N | Sex | Age | Presenting Symptom | CVD History or Risk Factor | Type of Non-Compaction | Associated Cardiovascular Anomalies | Genetic Study | Cardiac Magnetic Resonance Imaging | Treatment | Final Outcome |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 47 | Easy fatigability and mild hypertension since two years ago | HTN | NCLV, reduced LVEF (45%) | Coarctation of aorta | + | + | Carvedilol 6.25 mg TID plus spironolactone 25 mg daily | Good conditions |
| 2 | M | 56 | Progressive dyspnea | − | NCLV, LVEF = 16%, Global hypokinesia |
Hypoplasia of ascending and arch of aorta plus dilated main pulmonary artery | + | − | Scheduled for a valve-sparing aortic root replacement surgery + post-op carvedilol 6.25 mg TID, furosemide 40 mg daily plus spironolactone 25 mg daily | Doing well |
| 3 | M | 37 | Acute retrosternal pain with radiation to both shoulders since three hours prior to admission | − | NCLV, LVEF = 55% | Aortic dilation and coronary embolism | + | + | Anticoagulation plus dual antiplatelet therapy for two weeks switched to lifelong warfarin | Doing well |
| 4 | F | 34 | Dyspnea, two hours after admission, she experienced sudden cardiac death, resuscitated successfully with no sequela | − | BVNC, LVEF = 45% | BAV, ostium primum atrial septal defect plus complete heart block | − | − | Single-chamber implantable cardioverter-defibrillator | No high ventricular rate for 4 months |
| 5 | F | 41 | Echocardiography after angiography | Positive family history for CAD | NCLV, LVEF = 55% | BAV and Arteria Lusoria | + | + | Nil, suggested being under cardiologist follow-up at home country | Did not refer for follow-up |
| 6 | M | 43 | Dyspnea on exertion and recently at rest | − | BVNC LVEF = 24% and fractional area change = 16% | BVNC, BAV, and proximal muscle weakness in lower extremities | − | − | Daily furosemide 40 mg, spironolactone 25 mg, losartan 25 mg and carvedilol 12.5 mg | He left the hospital and did not refer for a follow-up |
| 7 | M | 48 | Dyspnea on moderate exercise and easy fatigability for six months | − | BVNC, LVEF = 55% | BVNC, BAV, aortic stenosis, and dilated ascending aorta | + | Denied because of claustrophobia | Spironolactone 25 mg, carvedilol 12.5 mg daily | Doing well 2 months later. He did not return for a follow-up |
| 8 | F | 25 | History of palpitation referred for the echocardiographic assessment | − | NCLV, LVEF = 32% | BAV, dilated ascending aorta and top normal size main pulmonary artery | − | − | Referred for CMR | Did not refer again to our center |
| 9 | M | 37 | Atypical chest pain for a month | − | NCLV, LVEF = 55% | Medial-lateral directed BAV’s cusps | − | + | Nil | Not yet referred |
| 10 | M | 48 | History of worsening palpitation, and a suspected right atrial mass on echocardiography, reported by a cardiologist | − | NCLV, LVEF = 55% | BAV, highly redundant and oscillating Chiari network | − | − | Suggested referring to his cardiologist in charge for further management | Did not refer again to our center |
| 11 | F | 62 | Echocardiography before diagnostic angiography | Old MI, DM, HLP | NCLV, LVEF = 34% | BAV | − | − | Indicated for CABG according to the result of coronary angiography, she refused and left the hospital | Did not refer again to our center |
| 12 | M | 58 | History of palpitation and shortness of breath on heavy exercise | − | BVNC, LVEF = 55% | Dilated aorta | − | + | carvedilol; 6.25 mg BID | Doing well |
Abbreviations: BAV; bicuspid aortic valve, BVNC; biventricular non-compaction, CABG; coronary artery bypass graft surgery, CMR; cardiac magnetic resonance, CVD; cardiovascular disease, DM; diabetes mellitus, HLP; hyperlipidemia, HTN; hypertension, LVEF; left ventricle ejection fraction, MI; myocardial infarction, NCLV; non-compaction of the left ventricle, TID; three times a day, BID; two times a day.