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Cath data


% of oxygenated Hb. Pulm. Ven. Sat. = 98% (100%). Arterial O2 Sat. = PV sat = 98-100%. Mixed ven. O2 Sat = 75-80% (PA). A drop of 2% in arterial O2 sat. compared to PV Sat. = RL shunt

RA RV PA PAW LA (PV) LV SA : a = 2-6; V = 2-4 m = 3 (0-6) : 15-25 edp 0-5. : 15-25; diast. 6-10, m = 10-15. : a = 6-12, V = 8-14, m 6-10 (12) : a=6-12, V=8-14, m = 6-10 (12) : 90-120 / 0-10 (12) : 90-120 / 60-75 (70-85).

8 months old infant,O/E CHF, Cyanosis

Site SVC RA RV PA LA LV Aorta PV Pressure(mmHg) -a12v7 mean 9 74/8 68/34, mean48 a4v6 mean 4 94/4-6 94/60 mean 74 mean 8 What is the diagnosis? Saturation(%) 84 78 80 79 76 75 76 98

Supracardiac Total anomalous pulmonary venous connection

Non -Restrictive interatrial communication

Pulmonary arterial hypertension

Complete Mixing of SV return and PV return at atria level Classical Supracardiac
Equal Saturation in all cardiac chambers

Ao Saturation > PA Saturation

PA Saturation > Ao Saturation

Large ASD & VSD No sign. Difference

Total Anomalous Pulmonary Venous Connection

Cardiac malformation in which there is no direct connection between any pulmonary vein & left atrium, but all the pulmonary veins connect to right atrium or one of its tributaries. A PFO or an ASD is present essentially all persons who survive after birth.

Wilson Muller Lewis & Varco : 1st description in 1798 : 1st closed partial approach in 1951 : Successful open repair in 1956

Total Anomalous Pulmonary Venous Connection

Origin of anomalous connection
1. Drainage to right atrium 2. Drainage to right common cardinal system (SVC or azygous vein) 3. Drainage to left common cardinal system (Left innominate vein or coronary sinus) 4. Drainage to umbilical-vitelline system (Portal vein, ductus venosus, hepatic vein)

Entire pulmonary venous return drains into the right atrium, usually via a common pulmonary vein confluence, resulting in complete pulmonary and systemic venous mixing. Oxygenated blood reaches the left heart via an inter-atrial connection (i.e.,ASD, PFO). Mechanical or functional obstruction of the pulmonary venous return leads to cyanosis, acidosis, pulmonary hypertension, & congestion.

1. Pulmonary venous anatomy
1) Type : Supracardiac 45% Cardiac 25% Infracardiac 25% Mixed 5% 2) Pulmonary venous obstruction . Junction of connecting vein or compression of long narrow connect vein . Functional obstruction (restrictive PFO)

2. Chamber & septal anatomy

. LA & LV : small . ASD or PFO : small in 1/2, rarely no ASD or PFO

3. Pulmonary vasculature
. Increased arterial muscularity . Structural change

4. Associated condition
. PDA : 15% . VSD : occasionally . TOF, DORV, IAA : rarely

RV & PA pressure


Pulmonary venous obstruction PVR


With obstructed TAPVC PA pressure frequently exceeds Systemic pressure With no or mild obstruction Mildly to moderately elevated

Elevated more with

LV & SA pressure

Pulmonary venous obst Restrictive PFO

Usually normal Decreased with

Restrictive PFO Obstructed TAPVC with R-L duct (very high PAP)


Clinical features & diagnosis
1. Presentation . Critically ill infants during 1st few week of life . Unexplained tachypnea & unimpressive cyanosis . Metabolic acidosis : pulmonary venous obstruction 2. Examination . Not particularly overactive heart & unimpressive heart sound 3. Chest radiography . Normal heart size with diffuse haziness or ground glass if pulmonary venous obstruction . Large heart size with high pulmonary blood flow . Figure of 8, snowman configuration

TAPVC: Hemodyanamic Issues

Infradiaphragmatic, Very early presentation More cyanosis Pul edema

Mild or no obstruction PFO Restriction

Good SpO2 High Qp/Qs Presentation Infancy

Low PV sat
More CHF PDA: Large R-L flow PA decompresses Less Pul Edema PDA small: Pul edema,RV failure

Restrict SBF High PBF High Qp/Ps Good SpO2 CHF early Congestion of both syst & pulm venous circ if Resistance to PBF is high PAH

Surgical emergency Surgery at presentation

BAS followed by Surgery

Natural history
1. Incidence
. Relatively uncommon anomaly, 1.5~3% of CHD

2. Survival
. Unfavorable prognosis 50% survival in 3months 20% survival in one year . Usually have pulmonary venous obstruction due to long pulmonary venous pathway & a small PFO . Those who survive the first year of life usually have large ASD, no pulmonary venous obstruction

Indications for operation
Immediate operation in any neonate or infant whom are importantly ill with TAPVC Prompt operation in any 6-12 months old infant Advisable if severe pulmonary vascular disease has not developed in old patients (under 8 units)

Supracardiac type: Side to side anastomosis between common pulmonary venous sinus & LA.Vertical vein ligated.ASD closed with cloth patch. To RA: atrial septum excised,patch is sewn in such a way that the pulmonry venous return is diverted to LA. To coronary sinus:unroofing of CS,patch closure of ASD

Infracardiac: Vertical anastomosis made between common pulmonary venous sinus and LA.The common PV which descends vertically is ligated above diaphragm

Sutureless technique
Sutureless technique is for the relief of PV stenosis. A, Theincision is made into the left atrium and extended into both upper and lower PVostia separately. B, Suturing is begun in thepericardium just above the junction of the superior PV with the left atrium. C, A second inferior suture is started below theinferior PV and continued in the same manner to the left atrial incision to jointhe superior suture line.

Primary Sutureless Repair

Small size of the pulmonary vein is a major risk factor for later development of PVS after conventional TAPVD repair The acute anatomic benefit for the sutureless repair is that each vein is its own native size, without any suture material to cause an excessive inflammatory reaction or luminal compromise

Surgical results
1. Operative mortality: 5-10% in non-obstructive type 20% in obstructive type 2. Modes of death . Hypertensive crisis . Pulmonary venous stenosis 3. Incremental risk factors for death . Infracardiac drainage . Pulmonary venous obstruction . Poor preoperative state . Small size of pulmonary vein . Increased PVR
. Small left ventricle

4. Cardiac rhythm:atrial arrhythmias 5. Reoperation

. Anastomotic stricture (5~10%) . Pulmonary vein stenosis

1 day old ,TAPVC to Hepatic vein

Site SVC IVC HV RA RV PA DAo LA LV O2 sat 32 38 65 44 42 42 46 48 48 Pressure mmHg

m12 110/16 115/80 80/55 m8 82/10

32 yrs old /F presented with recent onset of palpitations

Site SVC RA RV PA LA LV Aorta Pressure(mmHg) -a10v7 mean 6 34/8 32/18, mean 24 a11v8 mean 10 120/10 126/70 mean 94 Saturation(%) 64 84 80 79 96 94 96

There is significant step-up in Oxygen saturation from SVC-RA Mildly elevated PA pressure
Diagnosis could be
ASD PAPVC to RA Coronary AV fistula to RA

8 yrs old child with mild cyanosis

Site SVC RA RV PA LA LV Aorta Pressure(mmHg) -a6v3 mean 4 128/6 16/8, mean 12 a5v8 mean 4 124/8 126/70 mean 94 Diagnosis? Saturation(%) 60.7 64.6 62.6 66 96 86 87

Data suggests
Nonrestrictive VSD Severe PS
Tetralogy of Fallot

Cyanosis is due to right left ventricular shunt

Systolic pr in RV=LV=Ao Step down at LA-LV level Acyanotic /pink TOF-inc PA pr

15 yrs old child with H/O exertional dyspnea

Site SVC RA RV PA LA LV Desc Ao Pressure(mmHg) -a12v7 mean 10 124/10 120/70, mean 88 a11v8 mean 10 120/10 126/74 mean 92 Saturation(%) 66 67 65 72 96 94 86


Data shows:
Systemic RV and PA pressure Presence of desaturation in aorta

Differential Diagnosis
Large ductus arteriosus Large aorto-pulmonary window
With severe PAH

5 yrs old child referred for bradycardia

Site SVC RA RV PA LA LV Ao Pressure(mmHg) -a10v6 mean 6 124/8 20/10, mean 14 a6v10 mean 8 24/8 126/74 mean 92 Saturation(%) 66 67 96 72 96 70 96


This hemodyanamic data shows

AV & VA discordance
Congenitally corrected transposition of great vessels

Complete Heart block is common (2% per year) in patients with corrected TGA
Cause of bradycardia