TaPVD.pptx

Congenital

1. Associated with ASD Asplenia ( usually with Other cardiac defects) Poly splena ( usually with Other cardiac defects)

2. Huge flow on RV and PA --  PVOD Obstruction to PV drainge Obstruction at Intra atrial communication - decrease systemic flow

3. Sites of drainge LIV – CS RA SVC – AZUGOUS Infradiaphargamtic ( Heptic , gastric v, portal IVC , Ductus venosus

4. The magnitude of of RA blood passes to LA or Rv depends on relative compliance of both Ventricles

5. Sites of obstruction V V as it pass between LPA and L t bronchus before terminating in LIV VV as it pass to terminate in SCV it passes between RPA and Right bronchus Localized Obstruction Infradiaphragmatic as it pass in oespg hitus or pass the right to drain in portal v ASD Schematic diagram. The arrow indicates the site of obstruction in the ascending vertical vein. Ao indicates aorta; CPC, pulmonary venous confluence; LA, left atrium; LV, left ventricle; PT, pulmonary trunk; RA, right atrium; RV, right ventricle; SP, spine; and SVC, right superior vena cava.

6. If without obstruction : AS Big ASD with mild cyanosis and Heart Failure Hyperactive RV acc P2 ----PVOD may develop Later on If with Obstruction : cyanosis , no murmur ,ACC p2 ,HF, PO, Heart is not hyperactive HF occurs in all with obstruction and in 2/3 without PVOD

7. Left, Chest radiograph revealed marked cardiomegaly with snowman appearance of increased pulmonary vascular markings. Shin D et al. Circulation . 2011;123:e612-e613 Copyright © American Heart Association, Inc. All rights reserved.

8. Echo Dilated RA ,RV PA Pulmonary Hypertension Small LV ASD ( size ) Dilated SVC or Coronary sinus Echo free space behind LA Abnormal VC in suprasternal view or Long V to diaphragm in subcostal view may be seen

9. Total Anomalous Pulmonary Venous Connection(TAPVC). Two days old baby boy. Prenatal diagnosis of suspected congenital heart disease.

10. Figure 1. Still frame from online-only Data Supplement Movie, in a high parasternal short axis. Grosse-Wortmann L , and Friedberg M K Circulation . 2008;118:e103-e104 Copyright © American Heart Association, Inc. All rights reserved. . Still frame from online in a high parasternal short axis. The left- and right-sided vertical veins are seen arising from the common confluence. The left-sided vertical vein is shown draining into the left innominate vein near the left internal jugular vein (arrow). The drainage site of the right-sided vertical vein into the left innominate vein is not shown.

11. Transthoracic echocardiography revealed a dilated right ventricle and right atrium (RA) and a large amount of pericardial effusion (PE), along with an atrial septal defect (ASD). Shin D et al. Circulation . 2011;123:e612-e613 Copyright © American Heart Association, Inc. All rights reserved.

12. Four-chamber view of the fetal heart showing the dilated right atrium and right ventricle. The arrow shows the pulmonary venous confluence, which is not communicating with the left atrium.

13. Pulsed Doppler flow in the right pulmonary vein showing the absence of the typical peaks during systole and diastole. Instead, there is continuous low-velocity flow of 33 cm/s suggesting obstruction.

14. CT angiogram. Shin D et al. Circulation . 2011;123:e612-e613 Copyright © American Heart Association, Inc. All rights reserved.

15. Cardiac Cath Pressures Increased PAP RAP RVP High wedge if with obstruction RAP > LAP if with small ASD Increased Sat in RA = Ao Step up at the site of drainage Systemic hypoxia

16. Delineation of 4 PV is important so selective angio in Rt PA and LT PA Dye dilation Tech

17. Best if with big ASD , No PV obstruction and PAP< ½ systemic Death in 1 st month in those with obstruction , those without may survive to 1 st year Systemic arterial sat is about 90% RA sat = AO sat Decrease saturation with decrease sat blood with PVOD and with Pul HTN with increase unsaturated blood as with exercise ( decrease IVC sat )

18. Natural History CHF Death in 1 st year With PV obstruction --- Death in 3 months HF in all with obstruction and in 2/3 without obstruction Best combination --- Big ASD with No PV obstruction

19. Medical and Surgery TTT TT of HF , vent Atrial sptostomy Surgery If with obstruction 4-6 months if without old children if with no significant PVOD

20. Supra cardiac Wide open channel to LA with closure of ASD and VV IF to CS make it drain to LA + closure of ASD If to RA -  excition of atrial septum and and put a patch in such way to direct PVR to LA If to IVC ligate the descending V and open the chamber to LA, closure of ASD

21. Infracardiac TAPVC: (A) Ligation of the descending vertical vein at the level of the diaphragm, incision of the posterior wall of the left atrium as well the central pulmonary venous confluence; (B) the pulmonary venous confluence and left atrium are anastomosed.