Comments on a case of left ventricular hypertrabeculation/noncompaction
Editor—We read with interest the article by Errando and colleagues1 about splenic rupture and haemoperitoneum in a patient with left ventricular hypertrabeculation/noncompaction (LVHT). The reported case and the review of the pathophysiology of LVHT, however, raises several questions. Regarding the case history, the cause of the fall would be of interest, as LVHT may be complicated by arrhythmias and neurological disorders which might provoke falls.2 It is unusual that the ECG of the presented case was normal as only 10% of LVHT patients have normal ECGs. Frequent ECG abnormalities in LVHT patients are tall QRS complexes (43%), ST/T-wave abnormalities (37%) and left-bundle branch block (20%). At present, there are at least three different echocardiographic definitions for LVHT reported in the literature.2–4 It would be interesting to know which was used in this case and the echocardiographic findings in relation to left ventricular size and systolic function of the presented patient. The concurrent medication of the patient would be of interest. As LVHT is frequently associated with ECG abnormalities and heart failure, anaesthesiologists should avoid the administration of cardiodepressive or arrhythmogenic drugs.An increasing number of mutations in various genes has been reported in association with LVHT.5 LVHT also occurs spontaneously and in a non-familial form. The pathogenesis of LVHT is unknown. That LVHT develops during embryogenesis is only one of several pathogenetic hypotheses. LVHT has been observed to develop post-natally and, in a single patient, to disappear after myocarditis.5
Whether LVHT is associated with an increased risk of thrombo-embolism is debatable. The frequency of thrombo-embolism was not increased in 62 LVHT patients compared with controls matched for age, sex and ventricular function.6
A transplantation rate of 50% in LVHT patients is extremely high. In our own experience only a limited number of LVHT patients require heart transplantation because of intractable systolic dysfunction.
As neuromuscular disorders might complicate anaesthesia and require specialist management, the association of LVHT with neuromuscular disorders is of special interest for the anaesthetist. When systematically screened by a neurologist, up to 80% of patients with LVHT show a neuromuscular disorder.2 Neuromuscular disorders so far found in LVHT patients were Duchenne and Becker muscular dystrophy, dystrobrevinopathy, myotonic dystrophy, myoadenylate-deaminase deficiency, glycogenosis, zaspopathy, mitochondriopathy, Friedreich ataxia, Charcot–Marie–Tooth disease, and Barth syndrome.
To summarize, in LVHT patients undergoing anaesthesia not only the cardiac function, but also the presence of a neuromuscular disorder should be considered. For, in emergency situations, as in the presented case, extensive neurological investigations may not be feasible; it is recommended that patients with LVHT should be referred earlier to the neurologist, as soon as the diagnosis of LVHT is established.
C. Stöllberger*
L. Segall
J. Finsterer
Vienna, Austria
*E-mail: Claudia.stoellberger{at}chello.at
Editor—We thank you for the opportunity to respond to the questions posed about our article on splenic rupture in a patient previously diagnosed with non-compaction of the ventricular myocardium (NCVM).1 We believe their comments complement our report and review of the pathophysiology of this rare disease.
First, the fall at home of the patient was accidental, without signs of heart failure or dysrrhythmia on arrival in hospital. The neurological symptoms developed later in hospital and were probably related to his hypovolaemic state. As the patient had been diagnosed with NCVM previously, we gave only a brief description of the diagnostic procedures. Thus information about other specialists involved was not offered. The diagnosis was made using echocardiography and cardiac magnetic resonance imaging following the criteria of Jenni and colleagues.7 Our report focused on the anaesthetic management, no additional echocardiographic measurements were made during this admission. The medication therapy taken by the patient, apart from that quoted in the article, was amiodarone 200 mg daily.
As stated in the report, the ECG showed no specific abnormalities and only isoelectric T-waves were observed in the V4–V6 leads. Other than reported blood test alterations, no changes were observed.
For induction of anaesthesia, we chose drugs with fewer cardiodepressive effects and no arrhythmogenic properties, for this haemodynamically unstable patient.
With respect to the genetics and embryogenesis of the disease, we have followed the references cited and their comments coincide with our discussion.
The high incidence of emboli has been established by several authors,3 8 9 mainly in the adult forms, but we agree that, in other case series, the incidence may be lower. This may be well in relation to the incomplete or ‘minor’ forms of the disease and asymptomatic cases.
The patient described did not have concomitant neuromuscular disorders. However, we routinely monitor the neuromuscular block in emergency patients. Although this does not exclude the cited diseases, it is important to check for muscular weakness at the time of extubation in these critically ill patients.
C. L. Errando
Valencia, Spain
E-mail: c.l.errando{at}carloserrando.com
References
1 Errando CL, Tatay J, Serrano-Romero A, Gudín-Uriel M, Revert M, Peiró CM. Splenic rupture and haemoperitoneum in a patient with non-compaction of the left ventricular myocardium. Br J Anaesth 2005; 95:358–61
2 Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002; 90:899–902[CrossRef][Web of Science][Medline]
3 Chin TK, Perloff JK, Williams RG, Kenneth J, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990; 82:507–13
4 Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36:493–500
5 Stöllberger C and Finsterer J. Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 2004; 17:91–100[CrossRef][Web of Science][Medline]
6 Stöllberger C and Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology 2005; 103:68–72[CrossRef][Web of Science][Medline]
7 Jenni R, Oechslin E, Schneider J, Jost CA, Kaufman PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86:666–71
8 Agmon Y, Connolly HM, Olson LJ, Khandheria BK, Seward JB. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr 1999; 12:859–63[CrossRef][Web of Science][Medline]
9 Ritter M, Oeschlin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997; 72:26–3[Abstract]
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