Failing right ventricle

Transcription

1 Failing right ventricle U. Herberg 1, U. Gembruch 2 1 Pediatric Cardiology, 2 Prenatal Diagnostics and Fetal Therapy, University of Bonn, Germany

2 Prenatal Physiology Right ventricle dominant ventricle 59% combined cardiac output Right ventricular impairment flow foramen ovale performance of the LV % biventricular cardiac output Rudolph 2001, Mielke, 2001

3 Definition failing right ventricle Severe fetal right ventricular failure: systolic impairment (contractility ) PA-Flow diastolic impairment - Tricuspid regurgitation - Doppler venous duct ± Umbilical arteries ascites/pericardial effusion/hydrops

4 Conditions with failing RV Myocardial Disease Volume / Pressure Overload Structural heart disease

5 Case I 27+4 RV RA RV TR LV LV CTCR 0,61 Cardiomegaly, RV dilatation, myocardial thinning

6 Case I 27+4 RV RV RA RV TR LV PA LV PA + arterial duct: antegrade flow Pulmonary regurgitation normal pulsatility venous duct 27+4.

7 Suspected: Uhl s Anomaly Definition: hypoplastic myocardium of the RV free wall Histology: apposition of pericardial & endocardial layers absence of musculature Fetal Echocardiography: RV Dilatation thinning of the anterior wall of the RV RV hypocontractility ± abnormal tricuspid valve Uhl, 1952; Uhl, 1996; Gerlis, 1993; Vaujois, 2014

8 Case I at 33+6 DA DV Functional pulmonary atresia Duct: retrograde Flow pericardial effusion ductus venosus: neg. atrial contractions 33+6

9 Prenatal diagnosis: Uhl s Anomaly CS, ventilated with NO and O2 RV + LV hypocontractil

10 Postnatal follow up Ductal dependend pulmonary circulation PGE2, adrenaline, NO, O 2 48 hrs later anterograde pulmonary perfusion

11 Follow up 3 weeks 6 years MRI no fatty infiltrations, dilated RV, thinning of RV myocardium

12 Uhl s Anomaly - outcome intrauterine progression: failing RV with hydrops Fetal or neonatal death Adult with partial Uhl; MRI postnatal Various presentation Fontan/1 ½ ventricle/biventricular arrhythmia partial forms in adults Vaujois, 2014, Takizawa 2009; Hebert, 2010, Gerlis 2003

13 Case II 36+6 CTCR 0,

14 RV non-compaction Dilated RV, prominent coarse trabeculations at the RV apex

15 Case I CTCR 0,

16 RV non-compaction Dilated RV Prominent coarse trabeculations Endocardial fibroelastosis normal non compaction Ursell, 2013

17 RV non-compaction Definition: abnormal excessive trabeculation Clinical: Cardiac dysfunction/heart failure/hydrops Arrhythmias Thrombi Endocardial fibrosis Isolated/associated with CHD Ursell, 2013 RV-EFE

18 Postnatal follow up day 1

19 Postnatal follow up 6/12

20 Not the same with biventriuclar non-compaction DD zu biventrikulärem Befall 28

21 Case III severe RV dysfunction 22+1 RV LV RA LA + functional pulmonary atresia + reverse flow in the duct + pericardial effusion + EFE RV Ductus venosus: reverse a Pulsatile umbilical vein

22 Case III severe RV dysfunction 28+2 Ventricular tachycardia SSW Ventricular rate 218/min, atrial rate 143/min Fetal hydrops - Cesarian

23 Postnatal course CS 28+ wks, 1300 g Severe biventricular impairment FS 12%

24 AV-Block 2:1 with VES p p p p p p p VES VES VES

25 Nurses: funny ECG Long QT-Syndrome Typ 2 AV Block II. RV Cardiomyopathy Pulmonary Stenosis ß-Blocker TdP

26 Pace-Maker (1300 g)

27 Ventricular aneurysm/diverticle Rare, RV<<LV Outpouching of the RV dependent on size conservative Thx Oloron, Ped Cardiol 2011

28 Pressure and Volume Load

29 Constriction of the fetal ductus arteriosus Arterial Duct 39% of the biventricular cardiac output via duct Ductal occlusion increased RV afterload Increased FO flow Increased pulmonary flow Rudolph 2001, Hofstadler 1996 Hashima 2012, Zielinsky 2013

30 Twin 1, 27+2 GA, Indometacin-Thx

31 Definition of constriction of arterial duct Turbulent flow in the duct Systolic velocity > 1,4 m/s Diastolic velocity > 0.3m/s PI <2.2 m/s Absent flow = total occlusion Tulzer 1991, Zielinsky 2013

32 Sonographic features in solated DA closure Severe RV Hypertrophy Tricuspid valve regurgitation Pulmonary valve regurgitation Gewillig, 2009

33 Outcome in isolated DA closure Fetal hydrops IUFD Premature delivery Relevant early and late morbidity Pulmonary hypertension Gewillig, 2009

34 Twin-Twin Transfusion Syndrome - TTTS imbalance of placental anastomoses in monochorionicdiamnotic twin pregnancies volume shift from donor to recipient Recipient: increased pre- & afterload Myocardial hypertrophy Diastolic dysfunction Cardiac failure 53

35 Preoperativ cardiac function

36 Postoperative cardiac function

37 Conditions with failing RV Myocardial Disease Uhl s Anomaly RV Non-Compaction Diverticula/Aneurysms Volume / Pressure Overload TTTS Ductal constriction Shunts: AV-malformations, ductus venosus agenesis, Structural heart disease Ebstein s Anomaly Absent pulmonary valve syndrome

38 Postnatale Adaptation Fall of pulmonary vascular resistance Morin, 1992, Soifer, 1989

39 Perinatal Management of Fetal right heart failure Optimize ventricular function Reduction of pulmonary vascular resistance RV pulmonary vascular resistance keep duct open until LR-Shunt NO, O 2, Ventilation LV +/- careful catecholamin thx Gain time

40 Predictors of perinatal outcome in fetal CMP Dysfunction of both ventricles p 0,01 Holosystolic AV-regurgitation p 0,002 Abnormal diastolic Function p 0,047 -DV neg a -UV Pulsation N=55 Predictors of perinatal outcome in fetal CMP Pedra,... Hornberger, Circulation 2002

41 Conclusion The course of the failing fetal RV is dependend on the the function of the remaining LV to obtain cardiac output cut off values to predict perinatal outcome are still missing postnatally, fall of RV afterlaod allows improvement of RV function wait and see

42 Thank you for your attention