TAPVC A CASE STUDY
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This case study describes the repair of total anomalous pulmonary venous connection (TAPVC) in a 50-day-old baby. TAPVC is a congenital heart defect where the pulmonary veins do not connect normally to the left atrium. The baby underwent surgery to reroute the pulmonary veins and close an atrial septal defect. In the postoperative period, the baby developed pulmonary arterial hypertension crisis, which was treated with reintubation, vasoactive drugs, and sildenafil infusion through a pulmonary artery catheter. The baby's condition gradually improved and was extubated again after 48 hours of ventilation support.
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TAPVC A CASE STUDY
- 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
- 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
- 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
- Infracardiac TAPVC: The common pulmonary vein drains through the diaphragm into the portal vein or ductus venosus. Reprinted with permission from Ref. [12].