Naslov
Different clinical manifestation of myocardial noncompaction in two newborn

Autori
Dasović-Buljević, Andrea ; Šarić, Dalibor ; Buljević, Bruno ; Mustapić, Željka ; Benjak, Vesna ; Huljev, Sanda ; Malčić, Ivan

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
J Perinat Med 33 (2005) Suppl. I / 2005 - Zagreb, 2005

Skup
7th world congress of perinatal medicine

Mjesto i datum
Zagreb, Hrvatska, 09.2005

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Myocardial noncompaction

Sažetak
Myocardial noncompaction is very rare condition characterized by an altered structure of the myocardial wall due to incomplete myocardial ontogenesis (arrest in normal endomyocardial embryogenesis). Clinical symptoms may appear at any age and include heart failure, arrhythmias (which predispose to sudden death), and cardioembolic events. The diagnostic method of choice is transthoracic echocardiography. We present two newborns with diagnosed myocardial noncompaction. We diagnosed noncompaction according to the criteria suggested by Jenni et al. and by Sulafa and al. prospective study of 15 children with noncompaction. The finding of multiple trabeculations and recesses on cross- sectional imaging, with the appearance of distinct compacted and noncompacted myocardial layers, low scale colour flow mapping the delineating the continuity of intertrabecular recesses with the ventricular cavity and a ratio of 2:1 (at first) and 1.9:1 (at second newborn) of the thicknesses of the noncompacted to compacted layers. At our first patient hypoplastic left heart syndrome was diagnosed prenataly. He died after Norwood I procedure due to heart failure at the age of 9 months. Second patient was born from uncomplicated pregnancy. Shortly after delivery he developed symptoms of the heart failure and noncompaction myocardial structure was noticed. We started treatment with diuretics, ACE inhibitors, low doses of digitalis, calcium inhibitors and antiagregation therapy. His general condition improved but his weight increased slowly. Psychomotor development is mainly within normal limits. Myocardial noncompaction is mostly noticeable at the apicoseptal segment. Ejection fraction maintained at 45-50% (fractional shortening 23-28%), but with depressed diastolic function and left atrium enlargement, pathological mitral valve motion and venooclusive pulmonary hypertension (pulmonary valve regurgitation 2.5 m/s, trivial mitral valve regurgitation 3.5 m/s, mild dilated vena cava inferior). Stenosis of the isthmus of the aorta with 25 mmHg gradient was also described. There were no arrhythmias during previously mentioned therapy and no criteria for Roifman syndrome. He is followed for 7.5 months. We also present the first echocardiographic record of the reptile myocardium which phylogenetic structure probably reflex human noncompaction myocardium

Izvorni jezik
Engleski

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