Adult Congenital Heart Disease Litigation

By Dr Leisa Freeman, Honorary Consultant Cardiologist

The incidence of all levels of congenital heart disease (CHD) remains at 7-12/1000 live births. The numbers of adults with CHD now significantly exceeds the paediatric population due to earlier recognition of the anatomical problem, better palliation (by surgery or percutaneous intervention) and improved cardiological surveillance to watch for both the natural history of the condition and the unnatural history – e.g. the consequences of the initial palliation(s). The 18 year survival for a child born with complex congenital heart disease in the 1960’s was 10% whereas a child born with the same complex disease in the 1980’s had a 50% 18 year survival. Now > 90% survive into adolescence and beyond.

Coarctation of the aorta was first repaired in 1945 and frequently patients were discharged as ‘cured’. However, it is now clear that late complications relating to the type of surgical intervention (viz restenosis with end-to-end anastamosis, aneurysm formation with Dacron patch) require continued surveillance. Moreover some patients are prone to premature coronary artery disease, persistent upper limb hypertension (despite relief of the coarctation) and ascending aortic dilatation – probably related to the bicuspid aortic valve present in at least 50%. As a result of the natural and palliated sequelae, late survival is still compromised.

Transposition of the great arteries was palliated by the atrial repairs of Mustard and Senning from the mid 1960’s. This has permitted good survival for at least 30 – 40 years but the systemic right ventricle ultimately fails. Baffle obstructions and leaks are now dealt with percutaneously but considerable morbidity occurs with intra-atrial re-entry tachycardias. These difficulties led to the development of the arterial switch in which the great vessels are transposed and the coronary arteries are reimplanted. As ever in palliated CHD, late complications of sub pulmonary obstruction, neo aortic regurgitation and ostial coronary stenoses need knowledgeable follow-up in people post arterial switch .

Recent case:

A 32 year old lady with previous Mustard type atrial repair for transposition of the Great arteries (TGA), had been lost to follow up at the age of 15 (when she should have been transitioned to an adult congenital service). A gynaecologist, who documents her previous congenital heart surgery, prescribes Clomid to assist conception without arranging maternal cardiology adult congenital heart clinic review. The Expert obstetric opinion avers that this was Breach of Duty. The patient is referred for consultant obstetric review at 12 weeks of gestation (fortunately a singleton pregnancy) but fails to refer for maternal cardiology adult congenital review.

The Claimant’s pregnancy care was very disjointed, lacked precise information in respect of a systemic right ventricle, which at 26 weeks of gestation was eventually recognised (on echo) to be severely impaired. This information was not shared with the obstetric team.  Systemic right ventricle (atrial repair TGA) falls into WHO Pregnancy risk class III – 15% risk of morbidity particularly at delivery and post partum. The obstetric anaesthetists were completely unaware that she had a systemic right ventricle nor of the extent of its impairment (coding her ASA II) nor that in consequence of her previous surgery that she had occluded the superior limb of the atrial baffle and all the SVC return to the heart was via the azygous vein to the IVC and inferior limb baffle pathway.

The Claimant develops pregnancy induced hypertension (no markers of pre-eclampsia) in the last 3 weeks of pregnancy and delivers (Ventouse assisted 2nd stage), is discharged home from the delivery suite. She is admitted 3 days later in severe heart failure (not post partum cardiomyopathy) and has been referred for consideration of heart transplantation.

Pre pregnancy counselling must occur to discuss the risks to mother and baby (including inheritance risk – which in dTGA is not significantly raised) and to plan the antenatal, delivery and postnatal management with consultant obstetric and anaesthetic colleagues.

Cardiology Experts

This is also vital in the inherited aortopathies (e.g. Marfan, Loeys-Dietz, Ehlers Danlos Type IV) but also in those in whom a dilated aorta has been serendipitously found.

Follow up in dedicated adult congenital heart clinic is required for most patients born with a congenital heart condition. Further intervention is likely in most moderate and complex adult congenital heart disease.  Arrhythmias are the most likely cause of attendance in the Emergency department – and those circulations dependant on Fontan/TCPC pathways to provide pulmonary blood flow, are at high risk for decompensation if not managed promptly. Adult congenital heart patients are at high risk of endocarditis with primary care and Emergency departments those most likely to fail to consider it in a differential diagnosis. The rise in adult survivors of congenital heart disease has led to an increase in clinical negligence cases due to inadequate understanding of the underlying cardiac problem when admitted or seen with other acquired conditions. A recent case involved failure to use a bubble filter on an IV line in a patient with Eisenmenger Syndrome (which allows right to left shunting) and thus paradoxical embolus.  Life expectancy predictions for quantum in young adults may be challenging when the survivors of the type of palliative surgery (e.g. arterial switch) are now only just entering their 20’s and 30’s.

About the Author:

Dr Leisa Freeman (FRCP, FESC, FHEA, FRCOG (Ad Eundum) is a Honorary Consultant Cardiologist, she established the adult congenital service at Norfolk and Norwich University Hospital in 1993. It was recognised by NHSE as a Specialist Congenital Cardiac Centre in 2018. She set up a joint obstetric/cardiology clinic in 2000 for adult congenital patients, which expanded as a regional service for congenital and acquired (spontaneous coronary artery dissection, PPCM, dilated cardiomyopathy, arrhythmias) patients – for which provision of service and publications in the speciality, she was made a fellow of the Royal College of Obstetricians and Gynaecologist (Ad Eundum honoris causa) in 2021. She has undertaken medico-legal work since 2004 – Claimants 40% and Defendants 60%. She has been instructed as joint expert by the Welsh Health Board and prepared many joint statements following discussion of Experts. She sits on the Council and Cases Committee of the MDU, as well as the Secretary of State for Transport’s cardiovascular committee (DVLA). She remains an honorary consultant cardiologist at the Norfolk and Norwich University Hospital as she retired from NHS practice in October 2021. She will revalidate in 2023 and is still accepting instructions.

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