The complications of cardiac surgery: a walk on the Dark Side? Prof Rik De Decker Red Cross Children s Hospital
CME Nov/Dec 2011 http://www.cmej.org.za
Why should you care? You are about to leave your office after a hectic Friday, and are looking forward to a quiet weekend off. Just as you reach the door, the phone rings, and your first instinct is to ignore it. But you answer it anyway. It is a Mrs Harmse, in a panic. Then you recall: you had sent her 8-month-old son, Karl, who has tricuspid atresia, to Red Cross Hospital 6 weeks ago. Karl had become very blue when his Blalock-Taussig shunt, inserted 7 months ago, blocked. This time, she says, he has had diarrhoea for 3 days, he has become very blue again, and his face is now horribly swollen! She reads from his discharge letter from RXH, which says something about a successful heart operation 4 weeks ago with a Glenn
Do you wonder.? Who is this Glenn bloke, and why did he make Karl s head swell up? Or do you suspect.? Karl s been dehydrated by his diarrhoea, so his Glenn shunt has thrombosed, causing poor flow to his branch PAs and SVC syndrome! I d better get him to RXH now!
What does cardiac surgery do? Cardiac surgery (and cath interventions) removes or palliates physiological processes that lead to morbidity or death but it replaces them with other, more benign ones, that may not be entirely innocent.
What are those (less innocent) processes? The incorrect perception by parents that The Operation has been done, and all is well...
The life-line of a child with tricuspid atresia SIGNIFICANT EVENTS Antenatal/Birth ADMISSIONS Clinical assessment ECG Echocardiography Cardiac catheterisation CT angiogram Non-cardiac investigations Pre-op medical management Surgery Post-op TOE Post-op ICU echocardiograms Post-op catheterisation Twice daily ICU rounds Twice daily ward rounds Discharge work-up Echo ECG Bloods CARDIAC CLINIC ECG Echo Clinical assessment ROUTINE VISITS REFERRAL TO ADULT SERVICE Teenage years
The unnatural history of post-op CHD recoarctation hypertension PA distortion shunt obstruction LA enlargement LV hypertrophy PA aneurysm RV failure arrhythmias liver failure. etc, etc
Some common misconceptions Heart surgery is corrective Late post-op complications are rare Parents usually know what is wrong with their child s heart Only cardiologists need to know about heart operations Paediatric cardiologists hate being disturbed
Large variety of operations - from simple to complex
Five operative categories - by their longer-term outlook 1. temporary palliation for defects that cannot be repaired initially PA BAND SHUNTS 2. operations for heart defects that are fully corrected at the first procedure COARCTATION TAPVD TGA 1. heart defects that might require further surgery or intervention after repair TETRALOGY COARCTATION 2. heart defects that will definitely require further surgery after the initial procedure HOMOGRAFT FOR PA/VSD 1. long-term palliation of uncorrectable lesions: functionally univentricular hearts GLENN & FONTAN SHUNTS
Temporary palliation for defects that cannot initially be repaired Blalock-Taussig shunts (BTS) Central shunts Pulmonary artery bands (PABs) Palliative operations provide symptomatic relief but leave the basic pathophysiology uncorrected. they may create the false impression that the operation has been done. the patient is often lost to follow-up, and the late post-op mortality is consequently very high.
BT shunts (and a central shunt) Right BT shunt Left BT shunt Central shunt Temporary palliation for defects that cannot initially be repaired
PA band Aorta MPA Aorta MPA LPA Temporary palliation for defects that cannot initially be repaired
Operations for heart defects that are fully corrected at the first procedure Patent ductus arteriosus (PDA) Coarctation of the aorta Atrial septal defect (ASD) Ventricular septal defect (VSD) Atrioventricular septal defect (AVSD) Total anomalous pulmonary venous drainage (TAPVD) Transposition of the great arteries (TGA) Anomalous left coronary artery from the pulmonary artery (ALCAPA)
End-to-end coarctation repair Operations for heart defects that are fully corrected at the first procedure
Aortic switch operation for TGA Operations for heart defects that are fully corrected at the first procedure
Aortic switch operation for TGA Operations for heart defects that are fully corrected at the first procedure
Heart defects that might require further surgery or intervention after repair Tetralogy of Fallot (TOF) PV replacement for Fallot by far the most common redo procedure in adult congenital heart surgery Transposition of the great arteries (TGA) Ebstein s anomaly Mr Tim Jones Adult CHD surgery
Heart defects that will require further surgery after the initial procedure Homografts Pulmonary atresia with VSD (PA/VSD) Truncus arteriosus Transposition with pulmonary stenosis (TGA/PS) Congenital aortic stenosis (AS) Left ventricular outflow tract obstructions (LVOTO) Permanent pacemaker (PPM)
Homograft repair (Rastelli) Heart defects that will require further surgery after the initial procedure
Homografts always spell trouble! They do not grow with the patient Complications include PS and PR aneurysm formation branch pulmonary stenoses calcification endocarditis RV failure They need surgical replacement every 5-10 years!
Long-term palliation of uncorrectable lesions: the Fontan circulation (TCPC) for functionally univentricular hearts Tricuspid atresia Double inlet left or right ventricle (DILV/DIRV) Univentricular heart (UVH) Hypoplastic left heart syndrome (HLHS)
The Glenn shunt Heart defects that will require further surgery after the initial procedure
The Fontan operation
The 10 commandments for a Fontan repair 33 years later Choussat A, Fontan F, Besse P (1977) Selection criteria for the Fontan procedure. In: Anderson RH, Shinebourne EA (eds) Paediatric cardiology. Churchill Livingstone, Edinburgh, Scotland, pp 559 566 1. Age > 4 years 2. Sinus rhythm 3. Normal systemic venous return 4. Normal right atrial volume 5. Mean pulmonary artery pressure <15 mm Hg It is clear from a historic perspective that total compliance with all criteria does not necessarily portend excellent long-term survival. I suggest the following single commandment: Thou Shalt Be Perfect! 6. Pulmonary arteriolar resistance <4 Wood units/m 2 7. Pulmonary artery aorta ratio >0.75 8. Left-ventricular ejection fraction >0.60 9. Competent mitral valve 10. Absence of pulmonary artery distortion Fontan Ten Commandments Revisited and Revised Stern H. Pediatr Cardiol (2010) 31:1131 1134
Post-op complications of heart surgery Early: usually not your problem Late wound sepsis pericardial effusion pleural effusion obstruction endocarditis arrhythmias cardiac failure liver failure protein losing enteropathy etc, etc
Specific complications Shunts PA bands Glenn Fontan Tetralogy PA/VSD blockage, seroma slippage, PR, branch PA stenosis stenosis, blockage, SVC syndrome pleural effusions, liver failure RV failure, arrhythmias PA stenosis, PR, RV failure
What scar is that? median sternotomy all bypass cases, Glenn, Fontan right lateral thoracotomy RMBTS left lateral thoracotomy LMBTS, coarctation, PAB mini sternotomy pericardial effusion left subcostal permanent pacemaker right minithoracotomy ASD repair
Cardiac cath interventions 1. PDA occlusion 2. PDA stenting 3. ASD occlusion 4. VSD occlusion 5. Atrial septostomy 6. Relief of PA stenosis 7. Relief of conduit stenosis 8. Coarctation of aorta stenting 9. Aortic valvuloplasty 10. Pulmonary valvuloplasty 11. RV outflow tract stenting 12. Opening obstructed shunts 13. Recruiting disconnected PAs 14. Creating or closing Fontan fenestrations 15. Occlusion of pulmonary AVMs 16. Occlusion of PAPVD 17. Occlusion of coronary cameral fistulae 18. Occlusion of carotid-jugular fistula 19. Stenting of Takayasu aortitis 20. Myocardial biopsy 21. Renal artery stenoses 22. Retrieval of foreign bodies 23. Pericardiocentesis
Panic In conclusion What is a GP to do? Treat the symptoms and signs as best you can Phone a friend talk to them frequently Maintain ownership of your patient shared care! Learn to echo! UCT PG DIPLOMA IN PAEDIATRIC CARDIOLOGY
Shared care? SIGNIFICANT EVENTS CARDIAC CLINIC Clinical assessment Medication (INR, ECG, Echo) ROUTINE VISITS REFERRAL TO ADULT SERVICE Antenatal/Birth Teenage years rik.dedecker@uct.ac.za
Thank you for your attention!
The cardiac surgeon s idea of a waiting list.. A very cute patient