Azienda Ospedaliera Universitaria Integrata Verona ISOLATED ANOMALOUS DEVELOPMENT OF MYOCARDIUM DURING FETAL LIFE: EXPERIENCE OF OUR CENTRE C.Sandrini *, L.Rossetti *, M.Rebonato *, M.A.Prioli *, F.Bettinazzi, G.B.Luciani ^, C.Vassanelli* * Pediatric Cardiology Unit, Division of Cardiology, Prenatal Diagnosis Centre, Division of Obstetrics and Gynecology, ^ Pediatric Cardiac Surgery Unit, Division of Cardiac Surgery, University of Verona, Verona, Italy.
FETAL CARDIOMYOPATHIES Rare conditions High intrauterine loss Evolutive behavior Different outcomes: from poor prognosis to complete recovery Involvement of right ventricle, left ventricle or both Different causes, specific etiology difficult to identify Available treatments for some types (before-after birth)
PRENATAL DATA patient year GE (weeks) indication diagnosis hydrops arrhythmia 1 2007 20 previous demise severe LV dysfunction yes no 2 2008 33 suspicious of CHD severe LV dysfunction no no 3 2012 33 suspicious of CHD DCMP, severe biventricular dysfunction. no no 4 2012 22 suspicious of CHD aneurysm of the LV no yes 5 2013 23 suspicious of CHD diverticulum of the RV no no 6 2013 33 suspicious of CHD severe dilatation of the RV, Ebstein-like tricuspid valve with severe regurgitation no no 27,33333333 POSTNATAL DATA newborn sex year weight at birth (g) diagnosis 1 female 2007 2010 noncompacted ventricular myocardium 2 female 2008 2400 noncompacted ventricular myocardium 3 unknown unknown unknown unknown 4 male 2013 3400 aneurysm of the LV with normal coronary anatomy 5 male 2013 3332 diverticulum of the RV 6 male 2013 2640 severe dilatation of the RV with thinning of the myocardium, tricuspid dysplasia 2756,4 FOLLOW UP newborn F/U (year) outcome weight (Kg) complications ecg EF LV volume RV volume valve function therapy 1 1 dead 4 dead while waiting for cardiac transplantation LV hypertrophy 20% + + +. severe MR. 2 5 alive 17 none normal 64% normal. mild MR ACE I 3 0 lost at F/U........ 4 1 alive 10 arrhythmia (spontaneous recovery of sinus rhythm) Incomplete RBBB 37% + +. mild MR ACE I, Bb, ASA 5 0,5 alive 5 none normal normal normal. none none 6 0,5 alive 4 none normal normal normal + moderate TR none 1,33
CASE 1 Prenatal evidence of severe biventricular dysfunction (GA 33 weeks), no fetal hydrops At birth: diagnosis of noncompacted ventricular myocardium Progressive improvement of ventricular function, up to complete normalization No postnatal heart failure Therapy: inhibitors of angiotensin converting enzyme
CASE 2 Woman, 36 years, first pregnancy No maternal exposure to teratogenic agents; no familiar history of CHDs Normal fetal karyotype; no other fetal malformations GA at diagnosis: 22 weeks Diagnosis: large normokinetic apical aneurysm of the left ventricle with preserved basal systolic motion
34 weeks: fetal supraventricular arrhythmia, no fetal heart failure Two days transplacentar therapy with digoxin and flecainide (stopped because of maternal electrocardiographic alterations) No more organized fetal arrhythmias Normal labor at 39 week + 3 days of GA, uneventful Weight at birth: 3,400 kg
At birth: confirmation of thinning and dyskinesia of the muscular free left ventricular wall at distal and apical segments, with a moderately preserved left ventricular systolic function Six months later: worsening of left ventricular systolic function, with a decreased ejection fraction and left ventricular volume at the upper level of normality Perinatal period: single episode of atrial flutter with atrioventricualr conduction 2:1, spontaneous return to sinus rhythm Therapy: B-blocker (propranolol), aspirin and inhibitors of angiotensin-converting enzyme
CONCLUSIONS Different and evolutive behaviours Importance of prenatal diagnosis (associated CHDs? Treatable causes?) Importance of perinatal management in a tertiary Centre Importance of clinical and instrumental follow up after birth
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