ADULT CONGENITAL HEART DISEASE AN UPDATE FOR CARDIOLOGISTS AND PRIMARY CARE PHYSICIANS

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1 ADULT CONGENITAL HEART DISEASE AN UPDATE FOR CARDIOLOGISTS AND PRIMARY CARE PHYSICIANS V.S. Mahadevan, MD, F.R.C.P. Director, Structural and Adult congenital Interventional Cardiology Program William W Parmley Endowed Chair in Cardiology Associate Professor of Medicine University of California, San Francisco DISCLOSURES Proctor for Edwards Sapien TF aortic valve USA and Europe Proctor for Melody Medtronic pulmonary valve Europe Proctor for Edwards Sapien pulmonic valve - Europe 1

2 ACHD A GROWING POPULATION Incidence of congenital heart disease % of all births About 80% of all CHD patients will survive into adulthood in the Western world There are now more adults with congenital heart disease than children in the Western world SURVIVAL IN ACHD PATIENTS WITH COMPLEX HEART DISEASE Gruetmann.et.al. Congenit Heart Dis. 2015;10:

3 FALLOT S TETRALOGY 1. Ventricular septal defect 2. Pulmonary infundibular stenosis 3. Hypertrophy of Right Ventricle 4. Overriding of the aorta across the VSD REPAIR OF THE RVOT IN FALLOT S 3

4 WHAT HAPPENS FOLLOWING FALLOT REPAIR 1085 patients undergoing ToF repair from at GOSH, London 152 total deaths of which 100 happen within first year post op year 189 patients underwent PVR In patients >35 years age with no PVR mild RVOT obstruction was evident (mean gradient 24± 13mmHg) Frigiola et.al 4

5 TREATMENT OF RVOT IN ADULT WITH REPAIRED TOF Surgical PVR used to be standard Transcatheter valve is now considered first line in many patients Melody valve has FDA approval for use in this position. Currently Edwards Sapien valve can be used for large annulus patients Stent fracture and endocarditis are concerns 5

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7 Clinical and Hemodynamic Outcomes up to 7 Years After Transcatheter Pulmonary Valve Replacement in the US Melody Valve Investigational Device Exemption Trial CLINICAL PERSPECTIVE by John P. Cheatham, William E. Hellenbrand, Evan M. Zahn, Thomas K. Jones, Darren P. Berman, Julie A. Vincent, and Doff B. McElhinney Circulation Volume 131(22): June 2, 2015 Copyright American Heart Association, Inc. All rights reserved. Bar graph depicting New York Heart Association functional class before transcatheter pulmonary valve replacement and at each annual follow-up interval through 6 years as a percentage of patients with evaluation at each time point. John P. Cheatham et al. Circulation. 2015;131: Copyright American Heart Association, Inc. All rights reserved. 7

8 Top, Kaplan Meier curves depicting cumulative survival, freedom from Melody valve explantation, and freedom from reintervention on the Melody valve over time. John P. Cheatham et al. Circulation. 2015;131: Copyright American Heart Association, Inc. All rights reserved. Surgical pulmonary valve replacement outcomes 8

9 TETRALOGY OF FALLOT - Late VT is very common Cullen et al JACC 1994; 23: hr ambulatory ECG n=86-45% had complex ventricular ectopy or VT - 12 year follow up. No relationship to outcome. Conclusion Non sustained VT is common in TOF Non sustained VT doesn t predict sudden death No indication for drug therapy MOST PATIENTS ARE ASYMPTOMATIC! Sudden death 6% over the long term 4.5% per 100 patient years Shinebourne EA et al Heart 2006; 92:

10 TETRALOGY OF FALLOT - When is VT dangerous? Multicentre study n=793 - Age at repair 8.2 +/- 8 years - Follow up /- 8.7 years 16 sudden deaths - Older age of repair - Increased QRS duration - Increased rate of change QRSD - No reoperation despite adverse hemodynamics Pulmonary regurgitation was the main hemodynamic substrate for sustained VT or sudden death! Gatzoulis et al Risk factors for arrhythmia and sudden cardiac death late after repair of Tetralogy of Fallot. Lancet 2000; 356: POINTS TO REMEMBER RVOT dysfunction with pulmonic regurgitation / stenosis is common in adults with Tof repair Often Asymptomatic Refer those with significant PR to specialist center for further assessment Ventricular tachycardia can cause SCD even in relatively asymptomatic patients 10

11 TRANSPOSITION OF GREAT VESSELS dtga is the more common and without intervention long term survival is not possible Previous repairs include, Atrial switch procedure Mustard or Senning procedure Rastelli procedure where the VSD is used to connect the aorta to the LV and a conduit is placed between the RV and PA Arterial switch is the most common operation currently 11

12 ADULT PATIENTS WITH ATRIAL SWITCH Arrythmias Brady and tachyarrythmias Systemic right ventricular function failure Systemic AV valve (Tricuspid valve) regurgitation Baffle leaks Exercise intolerance / desaturation with exercise Complications following Atrial switch procedures Khairy et.al, Mustard vs Senning repair for transposition, CIY, 2004, 284:92 12

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14 ADULTS WITH ARTERIAL SWITCH OPERATION Pulmonary artery / RV outflow stenosis Coronary stenosis can occur Incidence is overall low Aortic root dilatation is common, although significant dilatation is not common Left ventricular systolic function is usually well preserved ARRHYTHMIAS IN ACHD PATIENTS Loss of sinus rhythm common cause of hospital admission Arrhythmia (39%) is the commonest cause of SCD in kids with CHD 1 Arrhythmia is the commonest cause of emergency admission in post-op ACHD px 2 SCD (26%) is the most common cause of death in adults with ACHD 3 1 Polderman FN et al Int J Cardiol 2004;95: Kaemmerer et al. J Thorac Cardiovasc Surgery 2003;126(4): Oechslin EN et al Am J Cardiol 2000;86(10):

15 ARRHYTHMIAS Arrhythmias can be difficult to detect (IART vs NSR) Often Multiple/Recurrent Significant morbidity, resistant to drug therapy, challenging to EP Life style impact SVT most common, can be as hemodynamically significant as VT (A flutter with 1:1 conduction in young patients) VT/SCD TRANSPOSITION OF GREAT ARTERIES Arrhythmia free survival post atrial switch Actuarial arrhythmia-free survival in 283 patients surviving for 30 days after Mustard or Senning operations with simple or complex transposition of the great arteries. 10yrs 78% 20yrs 57% 25yrs 36% P Moons et al. Heart 2004;90: Copyright BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved. 15

16 Bradyarrythmias Common in patients with d-tga and CCTGA Incidence of about 1-2% per year of loss of sinus rhythm, heart block In patients with uncorrected AVSD eg. older Down s syndrome patients Patients with previous ablations Loss of AV synchrony may worsen ventricular dysfunction FONTAN CIRCULATION A direct connection of the systemic venous circulation to the pulmonary arteries Often used in patients with tricuspid atresia Previously the RA used as a pumping chamber classical RA-PA Fontan Now most patients get a total cavopulmonary connection as a staged procedure, i.e the SVC is anastomosed to the RPA and the IVC via a conduit to the PA 16

17 ARRYTHMIA IN THE FONTAN PATIENT 26 year old female Tricuspid atresia with RA-PA Fontan and previous atrial arrythmias Severe RA enlargement Presented to clinic feeling generally unwell and some palpitations 17

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19 ARRHYTHMIA MANAGEMENT Understand the anatomy Chemical cardioversion often not possible most patients on amiodarone already Before DC version make a plan for emergency pacing since sinus node dysfunction may occur Consider TEE with appropriate anaesthetic support prior to DC version Systemic ventricular function may be poor and hence be careful with the choice of drugs 19

20 ASD IN THE ADULT PATIENT Most common in secundum ASD although other types may occur Atrial arrythmia / AF may be the initial presentation Consider TEE to assess in detail in TTE not definitive Consider referral for closure in evidence of RV volume overload If right heart enlargement out of proportion to ASD size consider TEE or CT scan to assess for anomalous pulmonary venous drainge Percutaneous closure is first line treatment of choice in all secundum defects. Largest available device size is 38mm in the US If PHT is seen on echo then will need right heart cath assessment If PVR <3.5 and shunt L R consider for closure If PVR higher will need pul. vasodilator study to assesses reversibility 20

21 ATRIAL ARRHYTHMIAS AND ASD Atrial flutter/fibrillation Early correction(<40) reduces the risk Short term - 2/3 rd with PAF will improve after ASD closure PVI prior to device closure; Surgical Maze + closure if indicated Listen to the patient Often will tell you what to do!! 21

22 INFECTIVE ENDOCARDITIS 4% of admissions to ACHD unit Only 40% will have an identifiable predisposing event Strept/entero/staph/HACEK Unusual organisms more common (immunodeficient) May require longer period of Antibiotics Prosthetic graft material in the heart Check regarding use of prophylactic antibiotics PREDISPOSING CONDITIONS 22

23 HEART FAILURE Systemic right ventricles may progressively fail even in the young adult Usual management principles of heart failure apply in most cases No clear evidence for benefit for ACEI / ARB in systemic RV and Fontan patients at the present time Avoid excessive diuresis especially in the polycythaemic patient. 23

24 PULMONARY EMBOLISM/HAEMOPTYSIS Have a high index of suspicion especially in the patient with pulmonary hypertension and Fontan type of circuit CT PA is the investigation of choice Eisenmenger patients with hemoptysis can be difficult to manage Bleeding from collaterals may be amenable to embolisation CYANOTIC CONGENITAL HEART DISEASE Eisenmenger s syndrome Associated commonly with unrepaired VSD s, or other types of shunt Ebstein s anamoly Usually have a large PFO and R sided failure can present with increased R to L shunt and cyanosis. Pul.atresia and other single ventricle physiology patients with shunts 24

25 GENERAL PRINCIPLES IN THE MANAGEMENT OF THE CYANOTIC PATIENT ACHD PATIENT Do not perform venesection Low threshold to rule out pulmonary embolus if clinically indicated If sudden worsening of cyanosis consider acute occlusion of shunt/s Use bubble traps for infusions in patients with shunts Avoid systemic vasodilators Seek ACHD specialist advice Thank you 25