DOROTHY RADFORD
Survival Rates of Children with Congenital Heart Disease continue to improve. 1940-20% 1960-40% 1980-70% 2010->90% Percentage of children with CHD reaching age of 18 years
1938 First Patent Ductus Arteriosus ligated by Dr Robert Gross 7 year old girl. He was Chief Resident in Surgery. His chief was away on leave. He had practiced in autopsy room and animal labs. Successful. The girl lived into her 80 s. Gross & Hubbard JAMA 1939 Surgical ligation of PDA
CYANOTIC LESIONS - TETRALOGY of FALLOT /PULMONARY ATRESIA FIRST PALLIATIVE OPERATION: BLALOCK-TAUSSIG SHUNT 1944 ALFRED BLALOCK HELEN TAUSSIG
SURGERY FOR ASD 1952 Dr John Lewis closed an ASD in a 5 year old girl using hypothermia and inflow stasis SUCCESSFUL OPEN HEART SURGERY Gott V L -Ann Thorac Surg 2005
1955 CROSS-CIRCULATION SURGERY LILLEHEI Surgical correction of VSD, Tetralogy of Fallot & Atrio-ventricular Canal defect Lillehei: The first open-heart repairs. Ann Thorac Surg 1986
HEART LUNG BYPASS MACHINE Dr John Gibbon 1953 Stoney -Evolution of CPB: Circ 2009
1971 Dr Francois Fontan Diagram from original article 1929-2018 Repair of Tricuspid Atresia Fontan & Baudet: Surgical repair of tricuspid atresia. Thorax 1971
FONTAN CONCEPT Separate systemic & pulmonary flows: (a) connect systemic veins directly to pulmonary arteries (b) use single ventricle as systemic pump REQUIRE 1. Adequate preload 2.Low pulmonary artery pressures 3.Negative intrathoracic pressures for flow to lungs
SINGLE VENTRICLE - Various types of Fontan operation A. CLASSIC FONTAN B. LATERAL TUNNEL C. EXTERNAL CONDUIT
SEQUENCE FOR A TRICUSPID ATRESIA PATIENT Balloon atrial septostomy Shunts Fontan completion Hemi-Fontan
PREVIOUSLY UNTREATABLE LESIONS BECAME TREATABLE PALLIATED BUT NOT CURED Double inlet left ventricle with outflow chamber Unbalanced atrio-ventricular canal defect with hypoplastic right ventricle Heterotaxia syndromes -single V,TAPVD etc
HYPOPLASTIC LEFT HEART SYNDROME For years babies with this condition were untreatable. They would die as the ductus arteriosus closed.
HYPOPLASTIC LEFT HEART SYNDROME Norwood et al; J Thorac Cardiovas Surg 1981 NORWOOD PROCEDURE 1981 FIRST OF THREE STAGED OPERATIONS-FONTAN 3RD
ADULT CONGENITAL HEART PATIENTS
AUSTRALIA & NEW ZEALAND FONTAN REGISTRY Ongoing studies & reports
Number Living 1400 1300 1200 1100 1000 900 800 700 600 500 400 300 200 100 0 ECC LT AP Number of Patients Alive with a Fontan Circulation Greater than 1500 in ANZ Registry 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 2005 2004 2003 2002 2001 2000 1999 1998 1997 1996 1995 1994 1993 1992 1991 1990 1989 1988 1987 1986 1985 1984 1983 1982 1981 1980 1979 1978 1977 1976 Year
Number of Patients AGES of FONTAN PATIENTS IN ANZ FONTAN REGISTRY 500 465 450 400 412 350 300 284 250 200 150 140 100 50 54 0 <10 10-18 19-29 30-39 >40 Age Group WANTING TO CONSIDER PREGNANCY
FONTAN HAEMODYNAMICS PRELOAD CARDIAC OUTPUT IS DEPENDENT ON THIS. No pump on pulmonary side. Relatively low CO. PULMONARY VASCULAR RESISTANCE should be low SYSTEMIC VENTRICULAR FUNCTION
AVOID FONTAN PHYSIOLOGY Decreased filling pressure (hypovolaemia) Increased Pulmonary Vascular Resistance & Positive Pressure Ventilation(e.g. GA) Increased LA pressure (LV dysfunction & A-V valve regurgitation)
COMPLICATIONS - FONTAN OPERATION Arrhythmias Thromboembolism Liver disease Protein-losing enteropathy Systemic ventricular dysfunction Hypoxaemia with shunts Plastic bronchitis Pregnancy risks Anaesthetic risks
Pundi et al: 40 Year follow-up after Fontan op J Am Coll Cardiol 2015
CYANOSIS FONTAN FENESTRATION Some desaturation from fenestration Also from veno-venous collaterals
PRE-CONCEPTION WORK-UP ECHO FULL MEDICAL EXAMINATION EXERCISE STRESS TEST MRI OXIMETRY
PREGNANCY HAEMODYNAMICS Increased Cardiac Output Increased Heart Rate Increased Plasma Volume Increased End Diastolic Volume Decreased Pulmonary Vascular Resistance Decreased Systemic Vascular R.
PREDICTORS OF POOR PREGNANCY OUTCOME IN FONTAN PATIENTS OXYGEN SATURATION < 90% ON AIR NYHA CLASS III OR IV BEFORE PREGANCY SYSTEMIC VENTRICLE EJECTION FRACTION < 40% PREVIOUS CARDIAC EVENT- Pulmonary oedema / Arrhythmia/ CVA SEVERE A-V VALVE REGURGITATION PROTEIN LOSING ENTEROPATHY FAILING FONTAN- hepatomegaly /ascites Brickner: CV management in preg CHD. Circulation 2014
MEDICATIONS in FONTAN PATIENTS ANTICOAGULANTS ACE INHIBITORS DIURETICS ANTI-ARRHYTHMICS ASPIRIN or WARFARIN CHANGE to LMW HEPARIN CEASE USE LOWEST EFFECTIVE DOSE CEASE SPIRONOLACTONE BETA-BLOCKERS CONTINUE AHA: Management of Pregnancy in complex CHD. Circulation 2017
MATERNAL CONCERNS in Fontan Pregnancy SUPRAVENTRICULAR ARRHYTHMIAS 3-37% PREGNANCY RELATED BLEEDING 5-50% Post partum haemorrhage 14% HEART FAILURE 3-11% Decline in NYHA function THROMBO-EMBOLIC EVENTS HYPOXAEMIA REVIEWS NO MATERNAL DEATHS 1.Ropero et al; 2018 Circulation: Cardiovasc Qual Outcomes 2.Moroney et al; 2018 Obstetric Medicine
FOETAL OUTCOMES LIVE BIRTHS 45% MISCARRIAGES 45% ELECTIVE TERMINATIONS 7% PREMATURITY 60% SMALL FOR GESTATION 25% NEONATAL DEATH 5%
FONTAN DELIVERY OUTCOMES PREMATURE MEMBRANE RUPTURE 10-14% CAESAREAN SECTION DELIVERY 57% ( 42-79%) EMERGENCY C SECTION RATE WAS GREATER THAN USUAL RATE
CONCERNS FOR FONTAN PATIENTS HYPOVOLAEMIA INCREASED INTRATHORACIC PRESSURE POSITIVE PRESSURE VENTILATION
RECOMMENDATIONS CAREFUL PRE-CONCEPTION MEDICAL ASSESSMENT & COUNSELLING EARLY MANAGEMENT PLAN FOR MEDICATIONS SPECIALISED REGULAR MULTI-DICIPLINARY PREGNANCY REVIEWS THROMBOPROPHYLAXIS- LMW HEPARIN PLANNED EPIDURAL ANAESTHETIC PLANNED VAGINAL BIRTH & MONITORING or PLANNED CAESAREAN SECTION DELIVERY ANTIBIOTIC COVER POST PARTUM HOSPITAL OBSERVATION
My Christmas Miracle Courier Mail 1999