Ascot Cardiology Symposium 2014 Adult Congenital Heart Disease What should GP s know? Boris Lowe, MB ChB, FRACP Green Lane Cardiovascular Service

Transcription

1 Ascot Cardiology Symposium 2014 Adult Congenital Heart Disease What should GP s know? Boris Lowe, MB ChB, FRACP Green Lane Cardiovascular Service

2 Objective

3 1 Hyperlipidemia does NOT cause congenital heart disease

4 Q: How often do you see adults with congenital heart disease in your practice? Vote Now 1. Never.. I take annual leave when they make an appointment 11% 2. 1 patient / year.. then I take sick leave to see my psychiatrist 52% 3. 1 patient / month 36% 4. 1 patient / week 0% 5. Every day.. now that Lorde has popularized the cyanotic shade of lipstick 2%

5 2 Patients with congenital heart disease are surviving into adulthood

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7 Numbers and proportions of adults and children with congenital heart disease Severe CHD Atrioventricular canal defects Tetralogy of Fallot Univentricular heart Transposition complex Truncus arteriosus Hypoplastic left heart syndrome Severe congenital HD ~10% of all congenital HD Marelli AJ, et al. Circulation 2007;115:163

8 Prevalence of congenital heart disease among patients of different age groups Median age (2000) 17 years

9 Pediatric to adult congenital heart disease Increased midterm survival Expanding population of adolescents and adults with congenital HD Fetal diagnosis Lower perioperative mortality Improved surgical and anesthetic techniques Early complete repair Advances in neonatal ICU care Increased early survival Incidence of congenital HD

10 Distribution of Congenital Heart Disease Diagnoses Van der Velde ET, et al. EurJEpidemiol 2005;20:549

11 3 Many adults with congenital heart disease can have a higher quality of life than their peers

12 345 patients (mean 26 ± 11 years) operated for isolated transposition of the great arteries, tetralogy of Fallot, and VSD. In all SF- 36 (health related QoL survey), and HADS (anxiety/depression aspects) health dimensions the GUCH patients showed excellent scores, which are comparable to the standard population, regardless of the initial CHD. 82% of patients asymptomatic 83% patients declared that they do not consider their QoL to be limited by their malformation

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14 Transposition of the great arteries, hypoplastic right ventricle, large VSD, severe pulmonary stenosis, Fontan procedure

15 Vote Now Q: Which of the following is TRUE regarding atrial septal defects? 1. ASDs cause a continuous heart murmur 13% 2. ASDs cause dilation of the right atrium, not the right ventricle 7% 3. An ECG is usually unhelpful to diagnose an ASD 17% 4. ASDs can present at any age 5. Quantifying a shunt fraction is required when deciding to repair an ASD 14% 49%

16 4 Atrial septal defects can present at any age

17 Atrial Septal Defects Common ~30% to 50% of children with congenital heart defects have an ASD as part of the cardiac defect. Ostium secundum ASD occurs as an isolated anomaly in 5 % to 10% of all congenital heart defects. Ostium secundum ASD 50-70% of all ASDs defect of fossa ovalis anomalous pulmonary venous return in ~10% of cases

18 ASD Physiology Oxygen rich blood flows across ASD from Left Atrium into the Right Atrium Enlarged Right Ventricle Enlarged Pulmonary Artery with increased blood Flow The magnitude of and direction of flow through an ASD depends on the size of the defect and the relative diastolic filling properties of the left and right ventricles. Significant L to R shunt when Qp:Qs >1.5:1 or right heart dilation.

19 Clinical Presentation No overt symptoms in many patients Exertional fatigue and dyspnea are the most common initial presenting symptoms Atrial fibrillation or flutter (onset usually >40 yo) Decompensated right heart failure ± pulmonary arterial hypertension (older patient) Tricuspid regurgitation Paradoxical embolus or TIA

20 Examination (Qp:Qs 1.5) RV heave ESM at ULSE (pulmonic) DM at LLSE (tricuspid) Widely- split S2 RBBB Loss or respirophasic variation PSM (MR, MVP) Loud P2 (PAH)

21 ECG Incomplete RBBB, rightward axis, RV hypertrophy

22 5 Congenital heart disease that has been repaired can still have late sequelae

23 Tetralogy of Fallot Subpulmonary Stenosis Overriding Aorta VSD Right Ventricular Hypertrophy

24 Tetralogy of Fallot Repair Infundibular stenosis resected VSD Closed with Patch Transannular Patch

25 Tetralogy of Fallot Repaired Survival % Years after Operation Unrepaired

26 Late Functional Sequelae Adverse functional sequelae at late follow- up after surgical repair of TOF : progressive exercise intolerance right heart failure symptomatic atrial and ventricular arrhythmia sudden cardiac death Etiological factors for these problems chronic pulmonary regurgitation (PR) right ventricular dilation and dysfunction myocardial scarring RV outflow tract obstruction (branch pulmonary artery stenosis) Redington AN, et al. Br Heart J. 1988;60:57; Carvalho JS, et al. Br Heart J. 1992;67:470; Zakha KG, et al. Circulation 1988;78:14.

27 Pulmonary regurgitation is associated with ventricular tachycardia and sudden death late after repair of tetralogy of Fallot Pulmonary Regurgitation Tricuspid Regurgitation RVSP >60mmHg Gatzoulis MA. Balaji S. Webber SA, et al. Lancet 2000;356:975

28 Indications for Pulmonary Valve Replacement Asymptomatic patients with 2 of: RV end- diastolic volume >150 ml/m2 RV ejection fraction <47% LV ejection fraction <55% Large RVOT aneurysm QRS duration >160 ms Sustained tachyarrhythmia Other significant abnormalities - severe branch pulmonary artery stenosis, moderate TR, septal defect Qp:Qs 1.5 Symptomatic patients with 1 of above Exercise intolerance Heart failure Syncope

29 Vote Now Q: Which of the following is TRUE regarding coarctation of the aorta? 1. Most patients with coarctation have a trileaflet aortic valve 2% 2. Surgical repair of coarctation of the aorta allows the discontinuation of antihypertensive medication 12% 3. A systolic blood pressure in the right arm 20 mmhg higher than the left arm is a classic examination finding 4. A common lesion in Down syndrome 14% 5. Notching of the anterior ribs can be seen on a chest X- ray 19% 54%

30 6 Hypertension can persist despite successful repair of coarctation of the aorta

31 Coarctation of the aorta Bicuspid aortic valve (up to 85%) Blood flow through the coarctation and PDA determines the blood flow to the descending aorta Significant coarctation peak pull- back gradient >20 mmhg gradient

32 Coarctation of the aorta repair

33 Late Sequelae Recoarctation Aneurysm formation following patch aortoplasty Late dissection and false aneurysms at the repair site Arm claudication of subclavian flap aortoplasty Residual hypertension (~ 1/3 patients by 20 years after operation) even in the absence of any residual gradient premature coronary artery disease cerebrovascular disease? Increased vascular resistance and hypoplastic arch upstream of CoA

34 7 Evidence-based treatments for left-sided heart failure are unproven for a systemic right ventricle

35 Systemic Right Ventricular Failure Progressive dysfunction of the systemic right ventricle is an inevitable complication in patients with Senning/Mustard procedures for complete transposition of the great arteries Congenitally corrected transposition of the great arteries The evidence base for the implementation of left ventricular failure treatment regimens in patients with a systemic right ventricle is poor Β- blockers could exacerbate pre- existing bradycardia and atrioventricular (AV) block Activation of the renin- angiotensin- aldosterone system could be a necessary compensatory mechanism in patients with pressure overloaded systemic RV

36 Systemic right ventricle β-blockers, ACE inhibitors and ATII antagonists trials

37 8 Antibiotic prophylaxis for infection endocarditis guidelines have been updated

38 Antibiotic prophylaxis recommendations Unrepaired cyanotic CHD, including palliative shunts and conduits Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization)

39 9 Psychosocial dysfunction of adults with congenital heart disease is common but frequently undetected

40 Psychological Distress Depression Anxiety Depressive symptoms 23% Elevated Trait Anxiety 33% Not depressed 77% Not Anxious 67% O Donovan C, Lowe B. CSANZ Young Investigator 2011

41 Causes of anxiety and depression Operations are often palliations, not cures Quality of life issues Exercise limitation Pregnancy / contraception Ongoing problems Rhythm abnormalities Heart muscle function Employment Insurance Reduced oxygen levels Endocarditis Possibility of reoperations Regular surveillance and follow- up

42 Making sense of their diagnosis helps

43 10 How do I refer a patient or contact an adult congenital cardiologist?

44 ACHD Clinic Card Green Lane Hospital Adult Congenital Cardiology Service Joseph A. Bloggs Diagnoses: iheart condition 1 iheart condition 2 iheart condition 3 Previous Surgery: Operation 1 Operation 2 Patient No. ABC1234 * Requires high risk protocol for endocarditis prophylaxis * Green Lane Adult Congenital Service Contacts: Dr. Tim Hornung Dr Clare O Donnell Dr Ivor Gerber Dr Boris Lowe Nurse Practitioner: Ms. Annette Rief Phone Fax annetten@adhb.govt.nz Out of hours call the Green Lane Hospital switchboard ( ) and ask for the on-call cardiology registrar

45 ACHD at Auckland City Hospital Cardiologists 2 paediatric (Hornung, O Donnell), 2 adult (Lowe, Gerber) 2 ACHD clinics and 1 pregnancy clinic weekly Nurse practitioner (Rief) Psychologist (Court) EP Cardiologist (Skinner) Surgeons (Finucane, Artrip) Anesthetists Inpatient care at Auckland City Hospital

46 Organization of ACHD in NZ General cardiology (large center) Regular ACHD clinic MRI ACHD outreach clinic Wellington Tauranga Auckland Christchurch ACHD Cardiology Cardiac Surgery Anesthesia Intensive care Catheterisation Transplantation Electrophysiology Cardiac MRI/CT Genetics Whangarei Hawkes Bay General cardiology (small center) ACHD outreach clinic

47 Fear Not Good life expectancy and quality of life is possible Diagnoses may be complex but modes of presentation are similar to non- congenital heart disease and other chronic diseases Most patients will not be discharged from tertiary ACHD Clinic follow- up