Download Adult Congenital Heart Disease

Ascot Cardiology Symposium 2014
Adult Congenital Heart Disease
What should GP’s know?
Boris Lowe, MB ChB, FRACP
Green Lane Cardiovascular Service
Objective
1
Hyperlipidemia does NOT cause
congenital heart disease
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Q: How often do you see adults with congenital
heart disease in your practice?
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% 2
Patients with congenital heart disease
are surviving into adulthood
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
Prevalence of congenital heart disease
among patients of different age groups
Median age (2000) 17 years Pediatric to adult
congenital heart disease
Expanding population of adolescents and adults with congenital HD Increased midterm survival Lower perioperative mortality Improved surgical and anesthetic techniques Fetal diagnosis Incidence of congenital HD Increased early survival Early complete repair Advances in neonatal ICU care Distribution of Congenital Heart
Disease Diagnoses
Van der Velde ET, et al. EurJEpidemiol 2005;20:549
3
Many adults with congenital heart disease
can have a higher quality of life than their peers
§  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 Transposition of the great arteries,
hypoplastic right ventricle, large VSD, severe
pulmonary stenosis, Fontan procedure
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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 49% 5. Quantifying a shunt fraction is required when deciding to repair an ASD 14% 4
Atrial septal defects can present at any age
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 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. 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 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) ECG
Incomplete RBBB, rightward axis, RV hypertrophy
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Congenital heart disease that has been repaired
can still have late sequelae
Tetralogy of Fallot
Overriding Aorta Subpulmonary Stenosis VSD Right Ventricular Hypertrophy Tetralogy of Fallot Repair
Infundibular stenosis resected VSD Closed with Patch Transannular Patch Tetralogy of Fallot
100 Survival % 80 Repaired 60 40 20 Unrepaired 0 0 5 10 15 20 25 Years after Operation 30 35 40 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.
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
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 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 54% 4. A common lesion in Down syndrome 14% 5. Notching of the anterior ribs can be seen on a chest X-­‐ray 19% 6
Hypertension can persist despite successful
repair of coarctation of the aorta
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 Coarctation of the aorta repair
Late Sequelae
§ 
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§ 
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 7
Evidence-based treatments for left-sided heart
failure are unproven for a systemic right ventricle
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 Systemic right ventricle
β-blockers, ACE inhibitors and ATII antagonists trials
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Antibiotic prophylaxis for infection endocarditis
guidelines have been updated
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) 9
Psychosocial dysfunction of adults with congenital
heart disease is common but frequently undetected
Psychological Distress
Depression
Anxiety
Depressive symptoms 23% Not depressed 77% Elevated Trait Anxiety 33% Not Anxious 67% O’Donovan C, Lowe B. CSANZ Young Investigator 2011
Causes of anxiety and depression
§  Operations are often palliations, not cures §  Ongoing problems –  Rhythm abnormalities –  Heart muscle function –  Reduced oxygen levels –  Endocarditis –  Possibility of reoperations §  Regular surveillance and follow-­‐up §  Quality of life issues –  Exercise limitation –  Pregnancy / contraception –  Employment –  Insurance Making sense of their diagnosis helps
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How do I refer a patient or
contact an adult congenital cardiologist?
ACHD Clinic Card
Green Lane Hospital Adult Congenital Cardiology Service
Joseph A. Bloggs
Patient No. ABC1234
Diagnoses: iHeart condition 1
iHeart condition 2
iHeart condition 3
Previous Surgery: Operation 1
Operation 2
* 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 0272271400
Fax 6310785
Email [email protected]
Out of hours call the Green Lane Hospital switchboard
(+64.96389909) and ask for the on-call cardiology registrar
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 Organization of ACHD in NZ
•  ACHD Cardiology •  General cardiology •  Cardiac Surgery (large center) •  Regular ACHD clinic •  MRI •  ACHD outreach clinic •  Anesthesia •  Intensive care Tauranga
•  Catheterisation •  Transplantation •  Electrophysiology Wellington
Christchurch
•  Cardiac MRI/CT •  Genetics Auckland
Auckland
•  General cardiology Whangarei
Hawkes Bay
(small center) •  ACHD outreach clinic 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