Cath Presentation

8/3/2019 Cath Presentation

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Cath data


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OXYGEN SATURATION (%)

% 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


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PRESSURES

RA : a = 2-6; V = 2-4 m = 3 (0-6)

RV : 15-25 edp 0-5.

PA : 15-25; diast. 6-10, m = 10-15.

PAW : a = 6-12, V = 8-14, m 6-10 (12)

LA (PV) : a=6-12, V=8-14, m = 6-10 (12)

LV : 90-120 / 0-10 (12)

SA : 90-120 / 60-75 (70-85).


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8 months old infant,O/E CHF, Cyanosis

Site Pressure(mmHg) Saturation(%)

SVC -- 84

RA a12v7 mean 9 78

RV 74/8 80PA 68/34, mean48 79

LA a4v6 mean 4 76

LV 94/4-6 75

Aorta 94/60 mean 74 76PV mean 8 98

What is the diagnosis?


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Diagnosis

Supracardiac Total anomalous pulmonary venous

connection

Non -Restrictive interatrial communication

Pulmonary arterial hypertension


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TAPVC

Complete Mixing of SV return and PV return atatria level

Classical Supracardiac

Equal Saturation in all cardiac chambers

Infracardiac

Ao Saturation > PA Saturation

Supracardiac PA Saturation > Ao Saturation

Large ASD & VSD No sign. Difference


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Total Anomalous Pulmonary Venous

Connection

DefinitionCardiac 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.

HistoryWilson : 1st description in 1798Muller : 1st closed partial approach in 1951

Lewis & Varco : Successful open repair in 1956


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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)


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TAPVC

Pathophysiology Entire pulmonary venous return drains into the

right atrium, usually via a common pulmonary vein

confluence, resulting in complete pulmonary andsystemic venous mixing.

Oxygenated blood reaches the left heart via aninter-atrial connection (i.e.,ASD, PFO).

Mechanical or functional obstruction of thepulmonary venous return leads to cyanosis, acidosis,pulmonary hypertension, & congestion.


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TAPVC

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

Morphology


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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

RVEDP Elevated more with

Pulmonary venous obst

Restrictive PFO LV & SA pressure

Usually normal Decreased with

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

TAPVC


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TAPVC

Types


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TAPVC

Clinical features & diagnosis1. Presentation

. Critically ill infants during 1st few week of life

. Unexplained tachypnea & unimpressive cyanosis

. Metabolic acidosis : pulmonary venous obstruction2. 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


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TAPVC: Hemodyanamic Issues

Obstructed

Infradiaphragmatic,

Very early presentation

More cyanosis

Pul edema

Low PV sat

More CHF

PDA: Large

R-L flow

PA decompresses

Less Pul Edema

PDA small:

Pul edema,RV failure

Mild or no obstruction

Good SpO2

High Qp/Qs

Presentation

Infancy

PFO Restriction

Restrict SBF

High PBF

High Qp/Ps

Good SpO2

CHF earlyCongestion of both

syst & pulm venous circ if

Resistance to PBF is high

PAH

Surgical emergency

Surgery at presentation

BAS followed by Surgery


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TAPVC

Natural history1. 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


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TAPVC

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)


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Surgery:corrective

Supracardiac type:

Side to side anastomosis between commonpulmonary 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


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Infracardiac:

Vertical anastomosis made between common

pulmonary venous sinus and LA.The common

PV which descends vertically is ligated above

diaphragm


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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 inthe same manner to the left atrial

incision to jointhe superior suture line.

Sutureless technique

TAPVC


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Primary Sutureless Repair

Rationale Small size of the pulmonary vein is a major risk factor for

later development of PVS after conventional TAPVDrepair

The acute anatomic benefit for the suturelessrepair isthat each vein is its own native size, without anysuturematerial to cause an excessive inflammatory reactionorluminal compromise


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TAPVC

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

Surgical results


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1 day old ,TAPVC to Hepatic vein

SVC 32

IVC 38

HV 65 RA 44 m12

RV 42 110/16

PA 42 115/80

DAo 46 80/55

LA 48 m8

LV 48 82/10

O2 sat Pressure mmHgSite


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32 yrs old /F presented with recent onset of

palpitations


SVC -- 64

RA a10v7 mean 6 84

RV 34/8 80

PA 32/18, mean 24 79

LA a11v8 mean 10 96

LV 120/10 94

Aorta 126/70 mean 94 96


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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


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8 yrs old child with mild cyanosis


SVC -- 60.7

RA a6v3 mean 4 64.6

RV 128/6 62.6

PA 16/8, mean 12 66

LA a5v8 mean 4 96

LV 124/8 86

Aorta 126/70 mean 94 87

Diagnosis?


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Data suggests

Nonrestrictive VSD

Severe PS

Tetralogy of Fallot

Cyanosis is due to rightleft ventricular

shunt


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TOF

Systolic pr in RV=LV=Ao

Step down at LA-LV level

Acyanotic /pink TOF-inc PA pr


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15 yrs old child with H/O exertional dyspnea


SVC -- 66

RA a12v7 mean 10 67

RV 124/10 65

PA 120/70, mean 88 72

LA a11v8 mean 10 96

LV 120/10 94

Desc Ao 126/74 mean 92 86

Diagnosis?


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Data shows:

Systemic RV and PA pressure

Presence of desaturation in aorta

Differential Diagnosis Large ductus arteriosus

Large aorto-pulmonary window

With severe PAH


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5 yrs old child referred for bradycardia


SVC -- 66

RA a10v6 mean 6 67

RV 124/8 96

PA 20/10, mean 14 72

LA a6v10 mean 8 96

LV 24/8 70Ao 126/74 mean 92 96

Diagnosis?


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This hemodyanamic data shows

AV & VA discordance

Congenitally corrected transposition of greatvessels

Complete Heart block is common (2% per

year) in patients with corrected TGA Cause of bradycardia

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Cath Presentation