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CASE STUDY TAPVC REPAIR 
JAYA BABU S 
STAFF NURSE 
CHICU
PATIENT PROFILE 
• 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
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 TYPE 
SYMPTAMATIC 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 
septal 
defect 
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). 
Decrease in 
pulmonary blood 
flow 
Decreased cardiac 
output, hypoxia, 
PAH CRISIS
RECOGNITION OF PAH CRISIS 
TACHYCARDIA 
High PAP 
ABRUPT DESATURATION 
HYPOTENSION 
BRADYCARDIA
Correct 
metabolic 
acidosis. 
PREVENTI 
ON 
Hyperventilate 
sedation 
Attenuate 
noxious 
stimuli 
Support 
cardiac 
output. 
100% 
oxygen. 
pulmonary 
vasodilators.
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….
TAPVC A CASE STUDY

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TAPVC A CASE STUDY

  • 1.
  • 2. CASE STUDY TAPVC REPAIR JAYA BABU S STAFF NURSE CHICU
  • 3. PATIENT PROFILE • 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
  • 4.
  • 5. 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
  • 6. TYPES SUPRACARDIAC 45% CARDIAC 25% INFRACARDIAC 25% MIXED 5%
  • 7. SUPRACARDIAC TAPVC PULMONARY VEINS CONVERGE BEHIND THE LEFT ATRIUM COMMON ANOMALOUS VERTICAL VEIN LEFT INNOMINATE VEIN
  • 8.
  • 9. CARDIAC TAPVC The pulmonary venous confluence drains into the coronary sinus
  • 10. INFRACARDIAC TAPVC The pulmonary venous confluence drains into a descending vertical vein through the diaphragm into the portal vein or ductus venosus.
  • 11. MIXED TAPVC It can involve any or all components of the previous three types.
  • 12. 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
  • 13. PATHOPHYSIOLOGY….. INCREASED PULMONARY BLOOD FLOW PULMONARY HYPERTENSION MUSCULARITY OF THE PULMONARY ARTERIOLES LABILE PULMONARY VASCULAR RESISTANCE
  • 14. 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
  • 15. OBSTRUCTED TAPVC … AT THE JUNCTION WHERE THE COMMON VEIN JOINS THE CORONARY SINUS AT THE ORIFICE OF THE CORONARY SINUS
  • 16. 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
  • 17. CLINICAL MANIFESTATIONS UNOBSTRUCTED TYPE ASYMPTAMATIC ONLY MILD CYANOSIS FAILURE TO THRIVE DYSPHONIA SLIGHT HEPATOMEGALY CARDIOMEGALY SYSTOLIC EJECTION MURMUR SNOWMAN SIGN IN C-XRAY
  • 18. OBSTRUCTED TYPE SYMPTAMATIC 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
  • 19. 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!!!!!!!!!
  • 20. Interrupt the connections with the systemic venous circulation An unobstructed communication between the pulmonary venous confluence and the left atrium close the atrial septal defect SURGERY
  • 21. SC TAPVC SURGICAL TECHNIQUE
  • 23. POSTOPERATIVE COMPLICATIONS • PULMONARY OEDEMA • PULMONARY HYPERTENSIVE CRISES • PHRENIC NERVE DAMAGE EARLY COMPLICATIONS • PULMONARY VENOUS OBSTRUCTION • ANASTOMOTIC STRICTURE • PULMONARY VEIN STENOSIS LATE COMPLICATIONS
  • 24. KEY POINTS IN POSTOPERATIVE CARE Maintain adequate cardiac output Keep the left atrial pressure as low as possible Prevention and management of PAH crises
  • 25. PA PRESSURE MONITORING • PAP should be than less than 2/3 rd of the systemic pressure • In PAH CRISIS, PAP becomes suprasystemic.
  • 26. Rapid increase in PVR PAP exceeds systemic blood pressure (BP). Decrease in pulmonary blood flow Decreased cardiac output, hypoxia, PAH CRISIS
  • 27. RECOGNITION OF PAH CRISIS TACHYCARDIA High PAP ABRUPT DESATURATION HYPOTENSION BRADYCARDIA
  • 28. Correct metabolic acidosis. PREVENTI ON Hyperventilate sedation Attenuate noxious stimuli Support cardiac output. 100% oxygen. pulmonary vasodilators.
  • 29. 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
  • 30. 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
  • 31. • 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
  • 32. • THE POSTOPERATIVE PERIOD IS CHALLENGING….

Editor's Notes

  1. Infracardiac TAPVC: The common pulmonary vein drains through the diaphragm into the portal vein or ductus venosus. Reprinted with permission from Ref. [12].