E. CARUSO 1, A. DI PINO 1, D. POLI 1, L. MANURI 2, P. PERCONTI 1, G. PONGIGLIONE 1, P. GUCCIONE 1 1 CENTRO CARDIOLOGICO PEDIATRICO DEL MEDITERRANEO, TAORMINA, ITALY, 2 POLICLINICO UNIVERSITARIO G.MARTINO, MESSINA, ITALY

Purpose Neonatal myocardial infarction (MI) is a rare event, with a hight mortality rate (90%). We describe two different cases of newborns with normal heart and normal coronary pattern, survived to myocardial infarction. Methods First case: a full term neonate presented with severe ashyxia after cesarean delivery. He was pale, hypotonic and polipnoic. He was treated with oxygen administration. The heart ratewas of 180 beats minute and the non invasive blood pressure was of 40/30 mmHg. Nasal cPAP ventilation was sterted and hypoglicemia and metabolic acidosis were corrected. One hour after admittance he presented a wide QRS tachycardia with a heart rate of 250 beats minute. The 12 leads electrocardiogram (ECG) showed a ventricular tachycardia (VT) (Fig1) with morphology of right ventricular outflow tract and inferior axis, that spontaneously resoved after few minutes. A trans toracic echocardiogram (TTE) showed a structural normal heart, normal origin of coronary arteries, severe global hypokinesia of the left ventricle (LV) ejection fraction (EF) of 10%, secon degree mitral regurgitation (MR) and mild tricuspid regurgitation with an estimated pulmonary artery sistolic pressure of 50 mmHg. Systemic blood flow was manteined by the persistence of a wide patent arterial duct (PDA) with a large shunt from the pulmonary artery and descending aorta. After some hours the ECG showed that ST tract was elevated in D1 mm and aVL and a marked ST segment depression from V1 to V6 (Fig 2). Baby was treated with low doses of inotropic infusional therapy and furosemide maintaining an adeguate cardiac output. Second case: a full term female neonate, born by normal vaginal delivery with birth weight 2.880 Kg and history of maternal smoking during pregnancy, referred at our institute for sospicious of myocardial ischemia at ECG. At admission she was hypotonic with erythrosic and dry skin, heart rate was of 150 beats minute and the non invasive blood pressure was of 50/30 mmHg. Blood examination showed a very hight haematocrit level (Htc) 80%, mebolic acidosis and normal C reactive protein, normal plateled count. Echocardiography showed hypocontractile LV with EF of 35%, moderate MR, trivial aortic regurgitation, small PDA with left to right shunt. The ECG showed inferior-lateral wall myocardial infarction (Fig 3-4). Infective agents were excluded at laboratory test and thrombophilia screening, urinary aminoacids and glycogenesis tests were negative. She was successfully treated with inotropic support, heparine e.v., dual oral antiplateded therapy (acetyl salicylic acid, 5 mg/Kg, and Clopidogrel, 0.2 mg/kg) and vasodilatators drugs. After 7 days she developed a severe myocardial hypertrophy, probably due to compensatory machanism. Conclusions Neonatal myocardial infarction is a rare event in the newborn with normal heart and normal coronary pattern, with very hight mortality. Thromboembolism, coagulopathy, birth asphyxia, erithroblastosis and enteroviral myocarditis are the most common causes. Early diagnosis and management in a tertiary centre is necessary. We describe two different cases of neonatal myocardial infarction. The first one had MI and VT after a birth asphyxia with natural arterial duct assistance and low drug assistance; it rapresents a rare case in term of treatment and long term follow up. The other one had MI after a severe secondary polycythemia, with very hight Htc, treated with medical therapy. MI and sudden death are more common in older patients with polycytemia and underlying coronary artery disease. Nimit C. et all described a case of 25 years old patient with spontaneous aortic thrombosis causing a left main coronary occlusion after secodary polycytemia. In neonates it is undescribed case. Both children are still alive: the first afrter 46 month's follow up, the second after 2 month's follow up.

S. OSSOLA 1, A. STIFANI 2, A. BIANCHI 1, M. AGOSTI 1 1 U.O. Neonatologia e TIN, Dipartimento materno-infantile, Azienda Ospedaliera Ospedale di Circolo e Fondazione Macchi, Varese, ITALY, 2 SSD Cardiologia Pediatrica, Dipartimento materno-infantile, Azienda Ospedaliera Ospedale di Circolo e Fondazione Macchi, Varese, ITALY

L'indice di perfusione (PI) č un indicatore continuo della perfusione sistemica e sembra essere un marker di gravitŕ delle condizioni cliniche da cause diverse. Esistono limitate segnalazioni sui valori di PI nella popolazione di nati pretermine, in cui puň verificarsi ipoperfusione periferica in presenza di pervietŕ del dotto arterioso (PDA). In questi pazienti la misurazione del PI potrebbe fornire un valido contributo non solo nel monitoraggio delle condizioni cliniche, ma nel guidare le scelte di trattamento del PDA, il cui bilancio rischio-beneficio č tuttora incerto. Obiettivi del nostro studio sono: 1) descrivere l'andamento dei valori di PI nei primi giorni di vita anche in correlazione con etŕ gestazionale (EG) e peso neonatale (PN); 2) validare l'utilitŕ del PI come marker di condizioni cliniche di maggiore gravitŕ; 3) valutare l'utilitŕ del PI come fattore predittivo di chiusura del dotto arterioso (DA), spontanea e/o dopo terapia minima. Materiali e Metodi Sono stati studiati 67 neonati di EG <32 settimane (EG 29,4 ± 2,2 settimane, PN 1204 ± 385 g). Sono stati suddivisi in base a: 1) EG in Gruppo 24-28 settimane e Gruppo 29-32 settimane 2) PN in Gruppo <1000 g e Gruppo >1000 g 3) supporto ventilatorio necessario in II giornata di vita in Gruppo Respiro Spontaneo (RS), Gruppo ventilazione non invasiva (Non Invasive Ventilation, NIV) e Gruppo ventilazione invasiva (Tubo EndoTracheale, TET) 4) modalitŕ di chiusura del dotto arterioso in Gruppo a chiusura spontanea, Gruppo a chiusura con singolo ciclo di ibuprofene, Gruppo a chiusura con piů cicli di COX-inibitore o legatura chirurgica. Il PI č stato misurato a 25-48, 49-72, 73-96, 97-120 ore di vita, in continuo per 10' (media di valori registrati ogni 20") alla mano destra e a un piede, mediante pulsossimetro Masimo Radical 7. Entro le 72 ore di vita i neonati sono stati sottoposti a ecocardiografia e monitorati quotidianamente fino alla chiusura funzionale del PDA. In caso di PDA emodinamicamente significativi con segni ecocardiografici di sovraccarico del cuore sinistro e/o ipoperfusione periferica sono stati trattati con COX-inibitori e/o legatura chirurgica seguendo le linee guida della Societŕ Italiana di Neonatologia Risultati In II, III, IV, V giornata di vita il PI mediano č rispettivamente 1,50; 1,76; 1,65; 1,68. I neonati di EG 24-28 settimane hanno PI medio tendenzialmente inferiore rispetto ai neonati di EG 29-32 settimane e i neonati con PN <1000 g hanno PI medio inferiore rispetto ai neonati con PN >1000 g (Fig. 1 e 2). A 25-48 ore di vita, il Gruppo RS ha PI significativamente maggiore rispetto al Gruppo NIV (1,99 ± 0,60 vs 1,46 ± 0,43, p = 0,002) e rispetto al gruppo TET (1,99 ± 0,60 vs 1,46 ± 0,45, p = 0,02) (Fig. 3). Il Gruppo a chiusura spontanea del DA ha PI maggiore rispetto al Gruppo a chiusura con singolo ciclo di COX-inibitore (1,75 ± 0,58 vs 1,50 ± 0,48) e rispetto al Gruppo a chiusura con piů cicli di COX-inibitore o legatura chirurgica (1,75 ± 0,58 vs 1,32 ± 0,43, p = 0,06) (Fig. 4). Conclusioni Il PI ha valori minimi in II giornata di vita, quando i neonati pretermine sono a maggior rischio di ipoperfusione periferica, ed aumenta nei giorni successivi, rispecchiando le modificazioni emodinamiche. Il PI ha una correlazione positiva con EG e PN e ha valori piů bassi nei pazienti in condizioni cliniche piů compromesse. Valori piů alti di PI in II giornata di vita sembrano essere predittivi di chiusura spontanea del dotto arterioso e pertanto la valutazione del PI potrebbe fornire un valido contributo nel guidare il trattamento del PDA.

C. RICCI 1, G. di salvo 1, C. morelli 1, F. fratta 1, L. di pietto 1, M.T. palladino 1, C. iacono 1, G. santoro 1, A. fusco 1, G. caianiello 1, P. argiento 1, M.G. russo 1 1 AO monaldi, napoli, ITALY

Introduction: Absent pulmonary valve syndrome (APV) is a rare cardiac abnormality characterized by the existence of a dysplastic or rudimentary pulmonary valve, associated in most instances with severe dilatation of the pulmonary trunk and its branches due to the concurrent occurrence of valve stenosis and regurgitation. The condition is generally associated with tetralogy of Fallot (TOF). Objective: The objective of this study was to assess the degree of accuracy of the prenatal diagnosis and to describe the characteristics, associations and outcome of APV diagnosed in the fetus in our third level center (AORN dei colli - AO Monaldi, Second university of Naples). Methods and results: Since January 1995 to April 2012 we practiced 7143 fetal heart scans. Among these, we diagnosed 1111 (16%) consecutive fetuses with CHD. An APV was diagnosed in 5 fetuses. The majority of patients were referred for a suspicion of congenital heart disease on obstetric scanning (4/5; 80%). Mean gestational age at diagnosis was 21 weeks. APV was found in association with TOF in all 5 cases. The karyotype, including 22q11 status, was known in all 5 cases. There were 4 (80%) isolated APV; 1 case (20%) was associated with 22q11 microdeletion. APV was associated with huge pulmonary regurgitation, enlargement of the infundibulum and of the pulmonary branches in all cases. There was 1 (20%) intrauterine death, due to fetal heart failure secondary to massive tricuspid regurgitation. The other four cases were livebirth. 2/4 (50%) babies were operated at a mean age of 7 months. These cases occurred in the last three years. The other two cases (50%) occurred before 2009 and they were operated sooner (mean age of 2 months). Surgical correction was obtained with biologic conduit between right ventricle and pulmonary branches bifurcation. 1/4 child died after surgery. She was operated at 7 months; she died for severe respiratory complications due to tracheomalacia. Three children are alive and well at a mean follow up of 5 years. Conclusions APV is a rare CHD. The diagnosis of an APV can be easily made by fetal echocardiography. The outcome for these patients largely depends on the presence of associated respiratory tract defects and their severity. Delayed surgical correction can be considered, if clinical conditions allow. Short-term (infant) outcome is relatively good.

G. ERRICO 1, R. BEGHINI 2, C. RICHELLI 2 1 Clinica Pediatrica Ospedale Policlinico G.B. Rossi, Verona, ITALY, 2 Terapia Intensiva Neonatale Ospedale Policlinico G.B. Rossi, Verona, ITALY

BACKGROUND: nei neonati pretermine, la persistenza del dotto arterioso (PDA) č una condizione molto frequente. Lo shunt sinistro-destro determinato dalla persistenza del dotto puň peggiorare il distress respiratorio ed aumentare il rischio di emorragia intraventricolare, di emorragia polmonare, di enterocolite necrotizzante e di malattia polmonare cronica. L'evoluzione di questa condizione puň essere la chiusura, spontanea oppure determinata dalla terapia farmacologica, o la sua persistenza che puň richiedere una correzione chirurgica. Il management di tale condizione rimane ancora discusso e controverso, soprattutto a causa dell'eterogeneitŕ dei criteri diagnostici impiegati per determinare la significativitŕ emodinamica del Dotto Arterioso. Uno studio condotto su neonati di peso inferiore a 1500 g durante la prima settimana postnatale ha evidenziato che uno shunt significativo era presente quando il diametro interno del dotto era oltre 1,5 mm.( Evans N, Iyer P. Assessment of ductus arteriosus shunt in preterm infants supported by mechanical ventilation: effect of inter-atrial shunting. J. Pediatrics, 1994;125:778-785). Un successivo studio sulla predizione ecocardiografica precoce di PDA sintomatico in neonati ventilati ha mostrato che a 19 ore di vita un diametro > a 1,4 mm, predice una successiva manifestazione clinica con 81% di sensibilitŕ ed 85% di specificitŕ. ( Kluckow M, Evans N. Early echocardiographic prediction of symptomatic patent ductus arteriosus in preterm infants undergoing mechanical ventilation. J. Pediatrics, 1995;127:774-779). Uno studio piů recente evidenziava come segno predittivo di " Dotto a rischio" il diametro del colletto all'eco-color Doppler >1,4 mm/Kg con sensibilitŕ pari a 94% e specificitŕ pari a 90% ( Hajjar, Arch Dis Child Fetal Neonatal 2005 ) SCOPI DELLA RICERCA: abbiamo cercato di individuare quale diametro interno del Dotto, indicizzato per la superficie corporea ( BSA ), sia predittivo di significativitŕ emodinamica in una popolazione di 25 neonati gravemente prematuri ( 18 ELBW con peso neonatale < 1000 gr e 7 VLBW con peso neonatale tra 1001 e 1500 gr ) METODI IMPIEGATI: abbiamo valutato con ecocardiografo Logiq 5 PRO General Electrics in asse corto parasternale sx in bidimensionale il diametro interno del Dotto di Botallo in 25 neonati pretermine ricoverati sequenzialmente presso la nostra Terapia Intensiva Neonatale da novembre 2011 ad aprile 2012 e l'abbiamo indicizzato per BSA di ciascun paziente calcolata al momento dell'ecocardiografia diagnostica, eseguita entro 48 ore dalla nascita. RISULTATI E CONCLUSIONI: il diametro interno medio del Dotto giudicato restrittivo č risultato essere <= 1 cm/mq, il calibro medio del Dotto trattato chirurgicamente era >=2,3 cm/mq ed il calibro medio del Dotto giudicato suscettibile di terapia farmacologica č stato pari a 1,1 cm/mq , nei Dotti responsivi ad un primo ciclo di terapia con Ibuprofene e pari ad 1,5 cm/mq nei Dotti responsivi ad un secondo ciclo di terapia con Ibuprofene. Per quanto riguarda I due gruppi ELBW e VLBW abbiamo osservato alcune significative differenze nelle due popolazioni. La chiusura spontanea del Dotto entro la 2° giornata di vita č avvenuta nel 25% degli ELBW e nel 42,8% dei VLBW. Il riscontro di restrittivitŕ del Dotto entro la 2° giornata di vita č stato pari a 12% negli ELBW ed a 28% nei VLBW. La necessitŕ di ricorrere a terapia chirurgica č stata pari al 20% negli ELBW ed a 0% nei VLBW. Le infezioni sistemiche sono la causa principale della ricorrenza di pervietŕ del Dotto nei pazienti , precedentemente trattati farmacologicamente con successo, ed osservata nel 29% dei casi trattati.

M. Tibaldi 1, F. Gabbarini 1, A. Villar 1, P. Murru 2, P. Gaglioti 3, A. Sciarrone 3, R. Bordese 1, G. Agnoletti 1 1 Divisioni di Cardiologia Pediatrica, Az. Ospedaliera OIRM- S. ANNA, Torino, Italia, 2 Divisione di Neonatologia, Az. Ospedaliera OIRM- S. ANNA, Torino, Italia, 3 Divisione di Ostetricia, Az. Ospedaliera OIRM- S. ANNA, Torino, Italia

INTRODUCTION Fetal tachyarrhythmia can be related to significant risk of morbidity and mortality. Supraventricular tachycardia (SVT) is the most common fetal tachyarrhythmia. There are 3 different possibilities of management: 1) close monitoring without therapy, 2) transplacental treatment, 3) early delivery. METHODS 9 fetuses with 1:1 atrioventricular conduction supraventricular tachycardia (SVT) and 2 fetuses with 2:1 atrioventricular conduction atrial flutter (AF) were evaluated during fetal life by echocardiography. Postnatal follow-up was done. In this study we excluded fetuses with isolated supraventricular extrasystolia and bigeminism.

Fetal diagnosis	GE	Transplacental Treatment	Fetal   follow-up	Neonatal diagnosis	Neonatal treatment	Postnatalfollow-up
SVT	30	Digoxin	Sinus rhythm	SVT	Propranolo/Flec	No relapse at 18 months 
SVT	34	no	SVT intermittent	Sinus rhythm	no	No relapse at 3 years
Atrial flutter2:1	33	Digoxin	Atrial flutter	Atrial flutter	Electrical cardioversion	No relapse at 3 years
SVT	39	No	Early delivery	SVT	Propranolol 1 year	No relapse at 3 years
SVT	35	No	Early delivery	SVT	No	No relapse at 3 years
SVT	16	Digoxin 		SVT	No	No relapse at 2 years
SVT	31	Digoxin, Flecainide	Sinus rhythm 	SVT	Flecainide 1 year	No relapse at 17 months
SVT, heart failure	23	Digoxin, Flecainide 	Fetal death	---	---	---
SVT	34	Digoxin 	SVT	Atrial Ectopic Tachycardia	Amiod/Flec/Propr	Atrial Ectopic Tachycardia at 16 months
SVT	28	Digoxin	SVT	SVT	Propr/Flec	SVT at 4 months
Atrial flutter2:1	34	Digoxin	Sinus rhythm	Sinus rhythm	no	No relapse at 2 years

RESULTS AND CONCLUSION In our experience fetal treatment was successful in majority of cases. Digoxin was used as first drug choice in all cases. In non-responding fetuses a second drug was added. There was one fetal death. Non - responding fetuses have in postnatal still tachycardia and we decided to control heart rate with therapy.

S. COSTA 1, E. PERVERSI 1, R. MAZZONI 1, U. BOTTONE 1 1 FONDAZIONE TOSCANA G.MONASTERIO, MASSA, ITALY

Background: Extracardiac malformations (ECM) represents an indication for fetal echocardiography and Congenital Heart Disease (CHD) can be present together with ECM in a single individual, however the frequency and the patterns of associations are not well defined. Aim: to evaluate in our experience the correlation between extracardial malformation as indication for fetal echocardiography and the presence of CHD in the fetus. Methods and Results: we performed a retrospective analysis in the 2010-2011 years: we examined 1684 pregnants with several indications to fetal echocardiography examination. We found 72 pregnants (4%) with extracardial malformation as indication; of whom 26 (36%) presented as indication single umbilical artery. In all fetuses we did not detect major CHD. Conversely we found 80 fetuses (4.7%) affected by major CHD in pregnant that showed as indication for fetal echocardiography suspected or detected CHD during morphologic echography. In particular we found, among them, 4 fetuses (5%) with ECM: one fetus with anomaly of the gastrointestinal and urinary systems, one case of aplasia cutis, one case with musculoskeletal anomalies and one case with anomalies in urinary system and tracheal malformation. We did not have any informations on the presence of ECM in 12 (15%) CHD fetuses because the pregnant have decided for a voluntary interruption of pregnancy. Conclusion: our data suggest that single umbilical artery as indication is not associated to the presence of major CHD in the fetus. However when a heart malformation is detected in a patient, a detailed investigation should be done on ECM.

D. Poli 1, E. Caruso 1, L. Manuri 2, P. Gitto 1, A. Baban 3, S. Agati 1, M. Saitta 1, P. Guccione 3, G. Pongiglione 3 1 Centro Cardiologico Pediatrico del Mediterraneo, Taormina, ITALY, 2 Policlinico Universitario, Messina, ITALY, 3 Ospedale Pediatrico Bambino Gesů, Roma, ITALY

Purpose Unbalanced atrio-ventricular septal defect with left hypoplasia is a very rare congenital heart disease and Down's syndrome is a risk factor in patients with functionally single ventricle Outcome of these patients is very poor and with hight mortality in the early period after Fontan procedure Methods We report a a case of monozygotic twins with Down's syndrome with unbalanced complete atrio-ventricular canal with left ventricular hypoplasia, both with muscular ventricular septal defects and one with persistent left superior vena cava Twins referred at our institute for diagnostic assessment and cardiac surgery Three months after birth they underwent to pulmonary artery banding and at age of one year they underwent to bidirectional Glenn leaving anterograde flow through the banded pulmonary trunk Conclusion This is an undescribed association of monozygotic Down's syndrome twins with 'non Down's syndrome atrio ventricular canal'

E. ROSATI 1, G. LATINI 2 1 Perinatal Cardiology Unit, Brindisi, ITALY, 2 Neonatology Division, Brindisi, ITALY

Prenatal detection of right ventricular hypoplasia (RVH) is a condition usually associated with pulmonary atresia with intact ventricular septum, tricuspid atresia or Ebstein's anomaly. Rarely, others cardiac anomalies leads to impairment of fetal RV development : prenatal diagnosis of these conditions can be difficult. We describe three rare cases of fetal RVH in presence of antegrade blood flow through tricuspid (TV) and pulmonary valve (PV), diagnostic features at birth and clinical follow up. Patient 1( GA 35 wks): fetus referred for RVH. Prenatal scan showed marked right atrial enlargement with normal TV z-value, mild TV regurgitation, the RV cavity slightly reduced. At birth oxygen saturation (SaO2) was normal, echo assessment confirmed prenatal features leading to diagnosis of idiopathic dilation of the right atrium (IDRA). After three years medical history was uneventful. Patient 2 (GA 21 wks): fetal detection of RVH and suspected pulmonary stenosis (PS). At birth cyanosis (SaO2 75%) and ductal-dependent pulmonary blood flow (PBF). Echo scan showed unobstructed PBF across the PV and a large membrane arising from the right atrium, protruding toward the RV and covering TV leaflets. There was a large foramen ovale with right-to-left shunt. Postnatal diagnosis was cor triatriatum dexter and membrane excision was successfully performed at 10th day of life. After three months,SaO2 raised to 94% and RV volume increased up to normal values. Patient 3 (GA 22 wks): fetal diagnosis of PS with RVH. Two hours after birth cyanosis (SaO2 80%), severe RVH and TV hypoplasia (6 mm; z-score - 3.7), normal flow across the PV, closed ductus arteriosus unresponsive to high doses of PGE1, large atrial septal defect with right-to-left shunt (isolated RVH). One month later SaO2 was stable, the baby was proposed for one and a half ventricular repair. In conclusion, fetal RVH can be caused by a rare conditions not easily detectable at prenatal scan. In these cases, clinical observation after birth is mandatory aimed to early detection of ductal-dependent PBF.

C. SANDRINI 1, L. Rossetti 1, A. Cristofaletti 1, M.A. Prioli 1, A. Ghidini 2, G. Lucchese 3, P. Santuz 4, C. Vassanelli 1 1 Cardiologia, azienda ospedaliera universitaria integrata, verona, ITALY, 2 Ostetricia e ginecologia, azienda ospedaliera universitaria integrata, verona, ITALY, 3 Cardiochirurgia, azienda ospedaliera universitaria integrata, verona, ITALY, 4 Pediatria, azienda ospedaliera universitaria integrata, verona, ITALY

BACKGROUND: The so-called "extended-basic scan" of the fetal heart, introduced in Italy in 2006 as screening for congenital heart disease (CHDs), improves prenatal diagnoses of congenital heart diseases. The study of the outflow tracts of the heart allows diagnoses of conotruncal malformation, that are not visible at the four-chamber view. AIM: The aim of this study is to assess the effects of the "extended-basic scan" of the fetal heart on antenatal diagnoses of CHDs in our Centre. METHODS: Between 2000 and 2010 2466 fetal echocardiographies were performed on 2090 pregnancies in our Centre. These were collected and divided into two groups (2000-2005 and 2006-2010). Both groups were analyzed and compared for diagnoses, indications, gestational age. RESULTS: The number of the diagnosed CHDs increased from 52 cases in the 2000-2005 period to 89 cases between 2006 and 2010. The number of malformations which proved to be detectable only through the study of the outflow tracts increased from 14/52 (27%) to 35/89 (39%). The indications for which patients were referred to fetal echocardiography changed in the period of time covered by the study. CONCLUSION: In our experience the introduction of the "extended-basic scan" of the fetal heart improves the detection rate of CHDs as it increases the number of antenatal diagnoses of conotruncal malformations. Team-work is essential for physicians (obstetrician, cardiologist, pediatric, pediatric cardiac surgeon) in order to correctly diagnose CHDs and to correctly manage newborns with heart malformations.

V. MARTUCCI 1, A. CAIARO 1, M. UNOLT 1, C. PUTOTTO 1, D. RUBINO 1, L.M. SILVESTRI 1, F. VENTRIGLIA 1 1 Dipartimento di Pediatria- Policlinico Umberto I- Sapienza- Universitŕ di Roma, Roma, ITALY

It happens ever so often that, for medicolegal reasons, obstetricians refer for fetal echocardiography (FE) pregnant women (PW) without risk factor. Is this echocardiographic screening useful and should it be routinely offered to all PW? In the period from 1st January 2011 to 30th March 2012, 1150 pregnant women asked to undergo their first FE at our Division of Pediatric Cardiology. We divided this cohort into 3 groups:
- Group A: 406 (35%) PW referred for screening
- Group B: 105 (9,5%) PW referred for ultrasound (US) suspicion of Congenital Heart Disease (CHD)
- Group C: 639 (55,5%) PW referred for family history of CHD, for genetic or chromosomal abnormalities, for pharmacological treatment during pregnancy or for other fetal or maternal indications. In the present study we focus specifically on the first two groups in order to compare them.
Group A: the maternal average age at diagnosis is 31,9 years (18-43), while the average gestational age (GA) at diagnosis is 27,4 weeks (19-38).
- 165 fetuses (40.5%) with normal cardiac findings;
- 216 fetuses (53.5%) with minor cardiac findings: 76 foramen ovale anomalies, 61 left ventricular false tendons, 41 valvular regurgitations, 9 interventricular septum (IVS) thickenings, 8 arrhythmias, 7 aortic arch (AA) anomalies, 5 enlargements of left/right heart chambers, 3 cases of tortuous ductus arteriosus (DA), 2 pulmonary artery dilatations, 1 Chiari's network, 5 other anomalies;
- 25 fetuses (6%) with CHD: 23 ventricular septal defects (VSD) mainly small and muscular, 1 Tetralogy of Fallot (TOF), 1 "borderline" left ventricle.
Group B: the maternal average age at diagnosis is 37,4 years (20-42), while the average GA at diagnosis is 32,2 weeks (18-36).
- In 34 fetuses (32,5%) FE confirmed the diagnoses suspected by US: 8 arrhythmias, 6 hypoplastic left heart syndromes, 5 VSDs, 4 atrioventricular canal (AVC), 2 right AA, 2 double outlet right ventricle (DORV) with transposition of great arteries (TGA), 2 pericardial effusions (PE), 1 TOF with Ebstein's anomaly, 1 right atrial enlargement, 1 TOF, 1 coarctations of aorta (CoAo), 1 ventricular disproportion (no CHD associated)
- In 33 fetuses (31,5%) FE disproved the diagnoses suspected by US: 15 arrhythmias, 7 VSDs, 6 cardiac tumors, 2 extreme levocardia, 1 TGA, 2 CoAo;
- In 21 fetuses (20%) FE disproved the diagnoses suspected by US but displayed other anomalies (eg. in TABLE 1)
- In 17 fetuses (16%) the US scan suggested an unspecified anomaly, that has been better defined by the FE (eg. in TABLE 2)
Conclusions:
- In the group of women referred for FE, 35% had no risk factors. Such large number of screening patients swell the waiting lists and in some cases delay the diagnosis of more serious diseases, which arrive at a definitive diagnosis only at a more advanced GA.
- Only 6% of the screening group had a CHD, mostly small VSD. However, non-diagnosed cases of aortic arch anomalies with severe LV dysfunction, as well as insidious truncoconal defects, such as TOF, have also been found.
- Despite the increased diagnostic awareness of obstetricians the margin of error is still very high (67,5%); in particular, low diagnostic accuracy and poor knowledge of the CHD follow-up result in erroneous first counseling which often affect the parents' decision.
In conclusion, we could propose two distinct levels of FE: a first level to screen the normal population before the fetal morphology ultrasound scan (about 20 weeks of GA) which would allow immediately for a correct counseling of fetuses with CHD; a second level exam, performed only in specialized centers, in order to arrive at a more precise diagnosis of CHD and to initiate the pre- and post-natal management of fetus with CHD.

CAIARO 1, M. UNOLT 1, V. MARTUCCI 1, C. PUTOTTO 1, F. VENTRIGLIA 1 1 Universitŕ La Sapienza - Dipartimento di Pediatria e Neuropsichiatria Infantile, Roma, ITALY

BACKGROUND: Anti-inflammatory drug, in particular the Nonsteroidal Anti-Inflammatory drugs (NSAIDs), have been reported to cause Ductus Arteriosus (DA) constriction in utero, and in some cases even to its premature closure during the fetal life. Recently some clinical trials have been initiated, in order to investigate the effectiveness of Paracetamol (PCM) in pharmacological closure of Patent DA (PDA) in preterm infants. Self-medication with this drug during pregnancy is very frequent and might cause fetal ductal constriction. In the clinical case that we are going to present, PCM intake during pregnancy seems to be the only risk factor for a severe fetal ductal constriction. CASE REPORT: We present the case of a 24-year-old caucasian patient, G1P0, that asked to undergo a screening Fetal Echocardiography (FE) in the 32nd week of pregnancy. She had a negative medical history, but she referred to have taken PCM 500/1000mg three times in one day. The FE showed a pronounced tortuosity of DA and Doppler mode showed increased systolic and diastolic velocities, up to 2 m/sec. We recommend not to take PCM and we referred the patient to obstetricians to control fetal flowmetry and monitor fetal heart rate, that resulted normal. The patient has been scanned again at our Department at 34 weeks gestation. The FE showed increased systolic and diastolic velocities, up to 2,9 m/sec and an enlargement of right heart chambers without tricuspid insufficiency. The patient referred to have taken PCM 500mg twice a day for three days. The patient underwent further periodic FE controls which showed increased systolic and diastolic velocities up to 3.24 m/sec and 1.08 m/sec respectively. The DA was pronouncedly tortuous and its pulmonary side was restricted. Beside the persisting right heart enlargement, the tricuspid insufficiency was observed. The left ventricle (LV) contractility was better than that of the right ventricle. The exam displayed also a hypertrophy of the interventricular septum (IVS) (5,8 mm) and a mild pericardial effusion (5 mm) behind the LV. On the basis of these finding we indicated an emergency caesarean section. The child was born at 36 weeks and 4 days, APGAR score 8-9 and body weight 2,970 kg. We performed a post-natal echocardiography (ECHO) during the first hours of life, that showed a large ductal ampulla near the aortic side of the DA. Increased tortuosity of the DA produced kinking. The restriction of the pulmonary side of the vessel worsened, as compared to the last FE, and the blood flow was further reduced. The minimal aorta-to-pulmonary artery shunting was intermittent. The exam displayed also an IVS thickness exceeding normal range (5 mm), a good global contractility and a patent foramen ovale (PFO) with modest bidirectional shunt. An electrocardiogram has been performed and showed non specific alterations of the repolarization phase. An ECHO control, performed in the 3rd day of life, displayed the spontaneous closure of the PDA and of the PFO. CONCLUSIONS: We have a strong clinical suspicion that PCM intake associated with a congenital ductal anomaly may have caused a significant DA constriction on its pulmonary side. This constriction continued to worsen, even though the patient stopped PCM intake. More cases are required to support this suspicion, but we can already suggest that gynecologists should advice patients, as they already do for other NSAIDs, against PCM self-medication during pregnancy and should recommend to take PCM only under medical prescription.

V. MARTUCCI 1, A. CAIARO 1, C. PUTOTTO 1, M. UNOLT 1, A. CEREKJA 2, G. BOSCO 1, R. LUCCHINI 1, G. PIACENTINI 1, F. VENTRIGLIA 1 1 POLICLINICO UMBERTO I SAPIENZA UNIVERSITA' DI ROMA, ROMA, ITALY, 2 ASL ROMA B, ROMA, ITALY

BACKGROUND: The fetal bradycardia (FB) is often an indication for emergency caesarean section. Usually FB is related to a benigne arrhythmia, such as blocked atrial bi/trigeminy, or a malignant one, such as complete Atrioventricular Block (AVB). Our clinical case suggests that this arrhythmia has to be regularly monitored in pre- and post-natal life, since it may cause Paroxystic Supraventricular Tachycardia (PSVT). CASE REPORT: We present the case of a 34 years old caucasian patient G1P0 that was referred to our department for "blocked atrial bigeminy with pseudobradycardia" detected elsewhere at 33 weeks gestation. The patient was scanned at our Department at 37 weeks gestation. An echocardiography showed during all the examination an arrhythmia that in M-mode resulted to be a blocked atrial trigeminy with a mean fetal heart rate (FHR) of 100 bpm . A female was born at 38 weeks. An ECG performed at birth revealed blocked supraventricular extrasystoles. An ECG the day after didn't registered any extrasystole. A day 3, an ECG showed no atrial extrasystoles and a QTc interval at upper limits of normal. An echocardiography performed the same day showed a small patent FO with moderate left to right shunt and a trivial tricuspid regurgitation. To our surprise, at day 11, ECG revealed supraventricular trigeminy and episodes of paroxystic supraventricular tachycardia. Therapy with Sotalol hydrochloride 2 mg/kg twice a day was started. At day 18, an episode of PSVT that needed a "diving reflex" maneuver was registered. The same day, a cardiomonitoring showed extrasystoles and episodes of bradycardia with a heart rate of 80 bpm. A 24hr ECG/Holter monitoring at day 20 recorded a sinusal rhythm with a mean heart rate at lower limits for age, some blocked supraventricular extrasystoles and 3 isolated and monomorphic ventricular extrasystoles. Actually, the little girl is administered Sotalol hydrochloride 2 mg/kg twice a day and she is doing well. CONCLUSION: Although most cases of fetal bradycardia verified during obstetrical ultrasound examination are due to vagal stimulation because of fetal compression by the transducer, attention should be paid in case of sustained bradycardias, trying to evaluate simultaneously atrial and ventricular contraction for evidence of nonconducted ectopic atrial beats causing low ventricular contraction rate or AVB. Furthermore, blocked atrial bi/trigeminy can present with bradycardia during the FHR monitoring leading erroneously to emergent preterm delivery. Consequently, in cases when an atypical bradycardia is registered at FHR monitoring, a fetal echocardiography should be done to determine if it is the case of blocked atrial bigeminy or trigeminy, because these arrhythmias do not represent an obstetrical emergency. In our case, in the first hours of life the fetal bradycardia due to atrial trigeminy has been confirmed and three subsequent postnatal ECG-s until day 3, showed no evidence of atrial extrasystoles, confirming the well-known frequent regression of this kind of benign arrhythmia. But at day 11 recurrence of supraventricular trigeminy and development of episodes of paroxystic supraventricular tachycardia were observed. Although our case represents a single evidence, it denotes the risk of development of more serious arrhythmia in postnatal life in cases of fetal blocked atrial ectopic beats. This seems to occur when the underlying mechanism is an accessory AV conduction pathway that appears occult even at basal ECG. On the basis of this observation, we recommend that fetuses with complex atrial ectopic beats should be closely monitored before and after birth for evidence of new arrhythmias. Since time period for monitoring is unknown, parents should be advised to verify baby's heart beat during feeding, quietness or sleep and refer both low and high frequencies.