Welcome to Journal of Cardiovascular Surgery and Heart Diseases

Keywords: Non-compaction; Cardiac involvement; Unclassified cardiomyopathy; Arrhythmia; Heart failure; Stroke

Letter to the Editor

In a recent article, Szulik et al. reported about a 22 years old female with ventricular fibrillation, QT-prolongation, and left ventricular hypertrabeculation/noncompaction (LVHT) who died from hypoxic cerebral damage 5 days after admission [1]. We have the following comments and concerns. Patients with LVHT have a disposition for any type of cardiac arrhythmia [2,3]. This is why ventricular fibrillation not only could be due to hereditary long-QT syndrome but also due to LVHT. Ventricular fibrillation was either due to LVHT or a consequence of QT-prolongation. QT-prolongation is not unusual in LVHT and has been reported in several cases (table 1) [3-7]. LVHT has been also reported in association with long-QT-syndrome due to mutations in the KCNQ1 gene [8], in the KCNH2 gene [9], or due to an unidentified genetic defect (table 1) [10]. In a study of 105 patients with LVHT, the QT-interval increased during a mean follow up of 3.6y in 15 patients and normalized in 21 patients [11]. The increase was associated with the extension of LVHT and the presence of a neuromuscular disorder (NMD) [11].

Since LVHT is associated with NMD in up to 80% of the cases [12], it would be interesting to know if the patient presented with any clinical features of a NMD or if the individual or family history was positive for a NMD. Additionally, it is of interest if the mother or any other relative had developed features of an NMD or had been diagnosed previously with a NMD. Second most frequently, LVHT is associated with chromosomal abnormalities. Was there any indication for numeric or structural chromosomal abnormalities? Since LVHT as well as sudden cardiac death (SCD) occurs familiarly, it would be interesting to know if the family was screened for cardiac disease after SCD of the index patient’s mother. In case the index patient had undergone echocardiography at the time of her mother’s decease, was LVHT detected already at this investigation? Were other family members screened for LVHT or ventricular arrhythmias? Did the index patient ever report palpitations, dyspnoe, leg edema, or exercise intolerance before the mother’s decease? Did the index patient undergo an echocardiographic investigation prior to her mother’s decease? Since LVHT is not only associated with arrhythmias and heart failure but also with stroke or embolism, it is also of interest if the index patient ever experienced an embolic stroke or peripheral embolism prior to the lethal event. Did her deceased mother ever experience a cardioembolic problem prior to SCD? Overall, this interesting case confirms that LVHT may be also associated with QT-prolongation and that associated ventricular arrhythmias may be fatal in some of these patients. In patients with SCD, relatives should be screened for LVHT and for severe arrhythmias. Implantation of an ICD should be considered in patients with LVHT and a family history positive for SCD. Treatment of OT-prolongation in LVHT depends on the cause, cardiac comorbidity, and duration of QT-prolongation.

References

1. Szulik M, Kukulski T, Kalarus Z, et al. 2014. Long QT syndrome and left ventricular non-compaction. Kardiol Pol. 72: 556.[Ref.]
2. Gerecke B, Engberding R. 2012. Isolated noncompaction cardiomyopathy with special emphasis on arrhythmia complications. Herzschrittmacherther Elektrophysiol. 23: 201-210.[Ref.]
3. Stöllberger C, Finsterer J. 2010. Arrhythmias and left ventricular hypertrabeculation /noncompaction. Curr Pharm Des. 16: 2880-2894.[Ref.]
4. Coleman MA, Bos JM, Phillips SD, et al. 2011. Left ventricular noncompaction syndrome masquerading or misdiagnosed as congenital long QT syndrome: remember QT prolongation does not equal long QT syndrome. Congenit Heart Dis. 6: 492-498.[Ref.]
5. Drago F, Stefano Silvetti M, Annichiarico M, et al. 2010. Biventricular pacing in an infant with noncompaction of the ventricular myocardium, congenital AV block, and prolonged QT interval. J Interv Card Electrophysiol. 28: 67-70.[Ref.]
6. De Rosa G, Pardeo M, Di Rocco C, et al. 2011. Neurogenic stunned myocardium presenting as left ventricular hypertrabeculation in childhood: a variant of Takotsubo cardiomyopathy? Pediatr Crit Care Med. 12: 420-423.[Ref.]
7. Rodrigues B, Correia E, Ferreira Santos L, et al. 2013. Left bundle branch block, atrioventricular block, torsade de pointes and long QT syndrome: is this too much for a rare cardiomyopathy? Rev Port Cardiol. 32: 425-430.[Ref.]
8. Nakashima K, Kusakawa I, Yamamoto T, et al. 2013. A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report. Heart Vessels. 28: 126-129.[Ref.]
9. Ogawa K, Nakamura Y, Terano K, et al. 2009. Isolated non-compaction of the ventricular myocardium associated with long QT syndrome: a report of 2 cases. Circ J. 73: 2169-2172.[Ref.]
10. Onay OS, Yildirim I, Beken B, et al. 2013. Successful implantation of an intracardiac defibrillator in an infant with long QT syndrome and isolated noncompaction of the ventricular myocardium. Pediatr Cardiol. 34: 189-193.[Ref.]
11. Stöllberger C, Gerger D, Jirak P, et al. 2014. Evolution of Electrocardiographic Abnormalities in Association with Neuromuscular Disorders and Survival in Left Ventricular Hypertrabeculation/Noncompaction. Ann Noninvasive Electrocardiol.[Ref.]
12. Stöllberger C, Finsterer J, Blazek G. 2002. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 90: 899-902.[Ref.]
13. Yoshinaga M, Ushinohama H, Sato S, et al. 2013. Electrocardiographic screening of 1-month-old infants for identifying prolonged QT intervals. Circ Arrhythm Electrophysiol. 6: 932-938.[Ref.]

Download Provisional PDF Here