Severe aortic regurgitation in a patient with left ventricular non-compaction cardiomyopathy

    Authors

    Keywords

    non-compaction cardiomyopathy, aortic regurgitation, heart failure

    DOI

    https://doi.org/10.15836/ccar2021.290

    Full Text

    Introduction : Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy characterized by excessive trabeculation and deep intertrabecular recesses most commonly affecting apical and mid-ventricular inferior and lateral segments. These patients are more prone to develop a typical triad of heart failure, thromboembolic events, and malignant arrhythmias. LVNC often coexists with other congenital or valvular heart diseases. Echocardiography is the standard imaging for the diagnosis of LVNC with several proposed criteria, mainly based on the ratio of non-compacted to compacted myocardial thickness ( 1 , 2 ). Case report: 75-year-old woman with a prior history of arterial hypertension and atrial fibrillation was hospitalized due to acute heart failure. Physical examination revealed holodiastolic precordial murmur, bilateral pulmonary crackles, and peripheral edema. Echocardiography showed severe regurgitation of tricuspid aortic valve based on the prolapse of the non-coronary cusp and volume overload – induced eccentric left ventricular (LV) hypertrophy with reduced ejection fraction (EF) of 35%. Moreover, a hypertrabeculation with intertrabecular recesses were observed in the inferolateral and apical region of LV fulfilling the echocardiographic diagnostic criteria for LVNC ( Figure 1 ). Coronary angiography showed no signs of coronary artery disease. The patient was started on standard heart failure therapy and was referred to cardiac surgery for aortic valve replacement (AVR). Postoperative echocardiographic assessment one year after AVR showed no improvement in LV systolic function despite normally functional bioprosthetic aortic valve and standard of care heart failure therapy. Four-chamber view showing ratio of the non-compacted and compacted myocardium. Conclusion : Symptomatic LVNC patients with LV systolic dysfunction generally have poorer prognosis ( 1 ). Since the potential of recovery of myocardial function is questionable in patients with preexisting myocardial disease ( 2 ), the postoperative outcome might not be satisfactory.

    Cardiologia Croatica
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    Severe aortic regurgitation in a patient with left ventricular non-compaction cardiomyopathy

    Extended Abstract
    Issue9-10
    Published
    Pages290
    PDF via DOIhttps://doi.org/10.15836/ccar2021.290
    non-compaction cardiomyopathy
    aortic regurgitation
    heart failure

    Authors

    Zrinka Planinić*ORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Petra Grubić RotkvićORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Jasna Čerkez HabekORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Marko PerčićORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Petar BešlićORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Edvard GalićORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia
    Jozica ŠikićORCIDUniversity Hospital “Sveti Duh”, Zagreb, Croatia

    Full Text

    Introduction : Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy characterized by excessive trabeculation and deep intertrabecular recesses most commonly affecting apical and mid-ventricular inferior and lateral segments. These patients are more prone to develop a typical triad of heart failure, thromboembolic events, and malignant arrhythmias. LVNC often coexists with other congenital or valvular heart diseases. Echocardiography is the standard imaging for the diagnosis of LVNC with several proposed criteria, mainly based on the ratio of non-compacted to compacted myocardial thickness ( 1 , 2 ). Case report: 75-year-old woman with a prior history of arterial hypertension and atrial fibrillation was hospitalized due to acute heart failure. Physical examination revealed holodiastolic precordial murmur, bilateral pulmonary crackles, and peripheral edema. Echocardiography showed severe regurgitation of tricuspid aortic valve based on the prolapse of the non-coronary cusp and volume overload – induced eccentric left ventricular (LV) hypertrophy with reduced ejection fraction (EF) of 35%. Moreover, a hypertrabeculation with intertrabecular recesses were observed in the inferolateral and apical region of LV fulfilling the echocardiographic diagnostic criteria for LVNC ( Figure 1 ). Coronary angiography showed no signs of coronary artery disease. The patient was started on standard heart failure therapy and was referred to cardiac surgery for aortic valve replacement (AVR). Postoperative echocardiographic assessment one year after AVR showed no improvement in LV systolic function despite normally functional bioprosthetic aortic valve and standard of care heart failure therapy. Four-chamber view showing ratio of the non-compacted and compacted myocardium. Conclusion : Symptomatic LVNC patients with LV systolic dysfunction generally have poorer prognosis ( 1 ). Since the potential of recovery of myocardial function is questionable in patients with preexisting myocardial disease ( 2 ), the postoperative outcome might not be satisfactory.