ISOLATED ANOMALOUS DEVELOPMENT OF MYOCARDIUM DURING FETAL LIFE: EXPERIENCE OF OUR CENTRE

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1 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.

2 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)

3 PRENATAL DATA patient year GE (weeks) indication diagnosis hydrops arrhythmia previous demise severe LV dysfunction yes no suspicious of CHD severe LV dysfunction no no suspicious of CHD DCMP, severe biventricular dysfunction. no no suspicious of CHD aneurysm of the LV no yes suspicious of CHD diverticulum of the RV no no suspicious of CHD severe dilatation of the RV, Ebstein-like tricuspid valve with severe regurgitation no no 27, POSTNATAL DATA newborn sex year weight at birth (g) diagnosis 1 female noncompacted ventricular myocardium 2 female noncompacted ventricular myocardium 3 unknown unknown unknown unknown 4 male aneurysm of the LV with normal coronary anatomy 5 male diverticulum of the RV 6 male 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 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

4 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

5 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

6 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

7 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

8 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

9 THANK YOU FOR YOUR ATTENTION