CASE STUDY TAPVC REPAIRJAYA BABU SSTAFF NURSE CHICU

• 50 DAY OLD BABY

• FIRST CHILD ,FULL TERM NORMAL DELIVERY

• BIRTH WEIGHT -2.7 Kg

• H/o bronchopneumonia at the age of 1 month

• Based on investigations and clinical symptoms detected to have SUPRACARDIAC TAPVC

• Underwent TAPVC rerouting

PATIENT PROFILE

TAPVC TOTAL ANOMALOUS PULMORAY

VENOUS CONNECTION

NO DIRECT CONNECTION BETWEEN ANY PULMOARY VEIN AND LEFT ATRIUM

ALL THE PULMONARY VEINS CONNECT TO RIGHT ATRIUM OR ONE OF ITS TRIBUTARIES

TYPES

SUPRACARDIAC

45%

CARDIAC

25%

INFRACARDIAC

25%

MIXED

5%

SUPRACARDIAC TAPVC

PULMONARY VEINS CONVERGE

BEHIND THE LEFT ATRIUM

COMMON ANOMALOUS

VERTICAL VEIN

LEFT INNOMINATE VEIN

CARDIAC TAPVC

The pulmonary venous confluence drains into the coronary sinus

INFRACARDIAC TAPVC

The pulmonary venous confluence

drains into a descending vertical

vein through the diaphragm into the

portal vein or ductus venosus.

MIXED TAPVC

It can involve any or all components

of the previous three types.

PATHOPHYSIOLOGY

• COMPLETE LA PV DISCONNECTION

• PV BLOOD GOING INTO RA

• AN INTRAATRIAL COMMUNICATION USUALLY ASD OR PFO

• DEGREE OF CYANOSIS DEPEND ON AMOUNT OF PULMONARY BLOOD FLOW

PATHOPHYSIOLOGY…..

INCREASED PULMONARY BLOOD FLOW

PULMONARY HYPERTENSION

MUSCULARITY OF THE PULMONARY

ARTERIOLES

LABILE PULMONARY

VASCULAR RESISTANCE

OBSTRUCTION TO PULMONARY VENOUS DRAINAGE

SUPRACARDIAC 65%

CARDIAC 17-20%

INFRACARDAIC 100%

SUPRACARDIAC

STENOSIS OF THE LEFT VERTICAL VEIN

COMPRESSION OF VERTICAL VEIN BETWEEN PULMONARY ARTERY AND

LEFT MAIN BRONCHUS

ANATOMIC VISE

OBSTRUCTED TAPVC …

AT THE JUNCTION WHERE THE

COMMON VEIN JOINS THE

CORONARY SINUS

AT THE ORIFICE OF THE

CORONARY SINUS

OBSTRUCTED TAPVC …STENOSIS WHERE IT ENTERS PORTAL OR HEPATIC VEIN

OR DUCTUS VENOSUS

COMPRESSION PASSING

THROUGH THE DIAPHRAGM

HIGH RESISTANCE PATHWAYS

IMPOSED BY FLOW THROUGH HEPATIC

MICROVESSELS

CLINICAL MANIFESTATIONS

UNOBSTRUCTED TYPE

ASYMPTAMATIC

ONLY MILD CYANOSIS

FAILURE TO THRIVE

DYSPHONIA

SLIGHT HEPATOMEGALY

CARDIOMEGALY

SYSTOLIC EJECTION MURMUR

SNOWMAN SIGN IN C-XRAY

OBSTRUCTED TYPESYMPTAMATIC WITHIN FEW

HOURS AFTER BIRTH

• MARKED RESPIRATORY DISTRESS WITH CYANOSIS

• FEATURES OF PULMONARY OEDEMA

• PROGRESS TO CARDIOGENIC SHOCK

• XRAY SHOWS MARKED VENOUS CONGESTION WITH A GROUND GLASS APPEARANCE AND NO CARDIOMEGALY

Medical management

UNOBSTRUCTED TYPE

• COMPENSATING RIGHT HEART FAILURE

– INOTROPIC SUPPORT

– DIURESIS

• OBSTRUCTED TYPE

LIMITED ROLE

INTUBATION AND HYPERVENTILATION

CORRECTION OF ACIDOSIS

OBSTRUCTED TAPVC IS A TRUE SURGICAL EMERGENCY!!!!!!!!!

Interrupt the connections

with the systemic venous

circulation

An unobstructed communication

between the pulmonary venous confluence and the

left atrium

close the atrial septaldefect

SURGERY

SC TAPVC SURGICAL TECHNIQUE

INFRACARDIAC TAPVC REROUTING

POSTOPERATIVE COMPLICATIONS

• PULMONARY OEDEMA

• PULMONARY HYPERTENSIVE CRISES

• PHRENIC NERVE DAMAGE

EARLY COMPLICATIONS

• PULMONARY VENOUS OBSTRUCTION

• ANASTOMOTIC STRICTURE

• PULMONARY VEIN STENOSIS

LATE COMPLICATIONS

KEY POINTS IN POSTOPERATIVE CARE

Maintain adequate cardiac output

Keep the left atrial pressure as low as

possible

Prevention and management of PAH

crises

PA PRESSURE MONITORING

• PAP should be than less than 2/3 rd of the systemic pressure

• In PAH CRISIS, PAP becomes suprasystemic.

Rapid increase in PVR

PAP exceeds systemic blood pressure (BP).

Decreased cardiac output, hypoxia,

Decrease in pulmonary blood

flow

PAH CRISIS

RECOGNITION OF PAH CRISIS

TACHYCARDIA

HYPOTENSION

High PAP

ABRUPT DESATURATION

BRADYCARDIA

PREVENTION

Correct metabolic acidosis.

Hyperventilate

sedation

Attenuate noxious stimuli

Support cardiac output.

pulmonary vasodilators.

100% oxygen.

POSTOPERATIVE COURSE

• SURGERY: Primary sutureless repair by right lateral approach.

• Received in ICU with stented sternum and PA line in situ.

• On ventilator with FiO2 80%– SIMV 32/TV-30/PEEP-4

– Ph-7.45 / Pao2-99.4 / Paco2-34.4 Lactate-0.9

• Stable hemodynamic– ABP:109/71 PAP:35/23(29) CVP:7

• Sedated with Morphine

• Milrinone 0.5mcg/kg/mt

POSTOPERATIVE COURSE Contd…• Lasix infusion 2mg/kg/day• Sternum closed after 24 hours• Extubated next day and put on NIV• Post extubation maintained stable hemodynamics and PAP within

normal range• Milrinone tapered off

• On th 4th POD ,PAP :69/38(50) ABP:54/32(41)– Spo2-93% ABG:7.35/ 68/44 – Lactate: 2.1– PERIPHERAL TEMP: 28.6 ,Urine Output –Nil for 3 hours

• Management:– Reintubated– Noradrenaline and Dopamine infusion started– Sildenafil infusion started in PA Line– PD started

• Improved clinically– ABP Improved

– PAP:24/20(22)

– Spo2 :100%

– Urine output 10ml/hr

• PD discontinued after 48 hours

• Supports were tapered off.

• Extubated again 48 hours of ventilation

• Put on NIV and O2 mask alternatively..

• Improving clinically

• THE POSTOPERATIVE PERIOD IS CHALLENGING….