ACHD & Heart Disease and Pregnancy: Guidelines and Cases Michael A. Gatzoulis

Transcription

1 ACHD & Heart Disease and Pregnancy: Guidelines and Cases Michael A. Gatzoulis Adult Congenital Heart Centre & National Centre for Pulmonary Hypertension Royal Brompton Hospital/National Heart & Lung Institute, Imperial College, London, UK

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4 Establishing the Diagnosis P < P < P < P < 0.001

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11 ACHD is a Chronic Heart Failure Syndrome MVO2 and Prognosis Diller et al Circulation 2005

12 PEAK VO2 PREDICTS COMBINED END-POINT OF HOSPITALIZATION OR DEATH Diller et al, Circulation 2005

13 DISTRIBUTION OF PEAK VO2 IN ASYMPTOMATIC ACHD PATIENTS (NYHA class I) Diller et al, Circulation 2005

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16 SCD is not a concern in: 1. Coarctation of the aorta 2. Tetralgy of Fallot 3. Transposition of great arteries and atrial switch procedure 4. Fontan circulation 5. Eisenmenger syndrome

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22 How can you differentiate between 2 ASD, 1 ASD and Sinus Venosus ASD?: 1. ECG QRS axis 2. ECG P wave axis 3. 2 nd heart sound 4. Pansystolic heart murmur 5. Cyanosis

23 Ventilation Q p/s O 2 carrying capacity Muscle Is ASD a right or left heart lesion? Webb & Gatzoulis Circulation 2007

24 CC closure for ASD: Exercise capacity 37 adults with ASD (mean 49 yrs) Exercise MVO2 prior to catheter closure and 6 months later Brochu et al, Circulation 2002

25 Closure of ASD: Reverse Remodelling Kort et al JACC 2001

26 < 40 yrs at repair (n=110) > 40 yrs at repair (n=103) Time from repair (yrs) Gatzoulis et al NEJM 1999

27 Murphy et al NEJM 1990

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29 Late complications of small VSDs are: 1. Atrial fibrillation 2. Endocarditis 3. Pulmonary arterial hypertension 4. Double chamber right ventricle 5. Left ventricular dysfunction One of these statements is incorrect

30 Case Study 34 years old female patient Came to seem me after her 3 rd successful pregnancy She had been treated for systemic hypertension since the age of 12 Having lived in 2 other major capitals of the world, before moving to London History of bronchial asthma

31 What single test would you like to perform? BP recording 2. Aldosterone and renin levels 3. Echocardiogram 4. CT 5. Other; please specify

32 Case Study Saw a respiratory physician, who ordered a CT Dx CoA At clinic with me had weak++ femoral pulses In retrospect, the UK midwife had noted absent femoral pulses BP Right arm 160/70 vs right leg 120/72mmHg

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34 247 adult patients with CoA, 33+/-13 years Assessed with CMR Re-stenosis in 31% (significant in 9%) Dilatation in 13% (discrete aneurysm in 9%) Restenosis likely after resection and end-to-end anastomosis Dilatation more likely after patch repair Systemic hypertension present in 69% Mortality over median FU of 5.9 years low, but higher than healthy controls Chen et al Int J Card 2014

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36 Chen et a Int J Card 2014

37 What is the likely diagnosis?

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39 Re-operation/s in TET (and timing) Criteria for PV replacement 1. CTR >65% 2. QRS prolongation 3. RVESV with severe PR 4. Reduction in MVO2 5. Symptoms (SOB, fatigue, arrhythmia) Babu-Narayan et al Circulation 2007 & 2014

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41 AICDs (Tetralogy patients, n=121) Secondary vs Primary Prevention Khairy et al Ciruclation 2008

42 Khairy et al Circulation 2008

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44 Dimopoulos Konstantinos PhD Imperial College 2008 Cumulative Mortality (%) CTR>55% CTR=48-55% CTR<48% 10 p< p< p< Patients at risk CTR>55% CTR=48-55% CTR<48% Time (years) Figure 41. Unadjusted survival curves according to cardiothoracic ratio Dimopoulos et al IJC 2013

45 Fontan Operation

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48 Fontan: TCPC Conversion/Transplantation? Indications and timing Mavroudis et al J Thorac Cardiovasc Surg 2001

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50 Case Study: Background 31 yo female patient Move to the UK in 2003 Previously diagnosed with a heart condition in Pakistan Started there on Nifedipine and Digoxin August 2003 admitted to her local hospital due to haemoptysis

51 Background SOBOE (1 flight of stairs) No chest pain, dizziness or black outs Sat (right arm): 95%, HR: 66bpm Normal S1 + split S2 Short LSB Respiratory examination unremarkable

52 Background Echocardiogram LV not dilated with good systolic function Normal function of the mitral and aortic valve RV mildly dilated, significantly hypertrophied, with preserved systolic function Significantly dilated pulmonary artery Mild TR, peak gradient 60 mmhg Positive bubble the atrial level (PFO)

53 What would you do next? 1. Right heart catheter 2. TOE 3. Cardiac MRI 4. CT scan 5. Refer to a respiratory physician, there is nothing wrong with the heart

54 Background V/Q scan Number of small bilateral defects partially matched in ventilation Traces in the kidneys suggesting R-L shunt CT- pulmonary angiogram Enlarged pulmonary arteries and RV No evidence of PE Some ground glass opacification at the LLL consistent with recent pulmonary haemorrhage

55 Background TOE RV dilated, significantly hypertrophied with preserved systolic function Positive bubble study, R-L shunt though a PFO Lung function test Normal volumes Slightly reduced gas-transfer

56 Blood test

57 6 min walk test 6 minute-walk test Pre-test Post-test Saturations 97 % 94% Heart rate Distance 270 m

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60 What would you do next? 1. It this idiopathic PAH? Start patient on advanced therapy 2. High resolution CT scan (DLCO is reduced) 3. The patients is iron deficient, which justify the symptoms, therefore, she only needs iron 4. Right heart catheter 5. I have no idea of what is going on

61 Establish the Diagnosis P < P < P < P < 0.001

62 Bosentan reduces pulmonary vascular resistance indexed 300 PVRi (dyn sec cm -5 ) Change from baseline Placebo (n=17) Bosentan (n=36) T.E. = dyn.sec.cm -5 p=0.04 Galie et al for Breathe-5, Circulation 2006

63 Bosentan increases exercise capacity 60 6MWD (m) Change from baseline Placebo (n=17) Bosentan (n=37) T.E. = 53.1 m p= Galie et al for Breathe-5, Circulation 2006

64 Survival benefits with advanced therapy No advanced therapies Advanced therapies 40 Cumulative mortality (%) Time (years) n n Cumulative mortality (%) p = Time (years) Dimopoulos et al Circulation 2010

65 PAH-CHD Groups and Therapy a. Eisenmenger syndrome b. Operated shunt (VSD) c. PAH with a small shunt d. PAH with L R shunt e. Fontan circulation Gatzoulis et al IJC 2014

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67 2 nd Edition Cambridge Publishing 2016

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71 Pregnancy counselling: Elective Risk of pregnancy Maternal death (background risk of 1:10,000 in the UK) Other potential complications to consider (than death): Stroke/Arrhythmia Overt Heart Failure Adverse effect on cardiovascular function and thus, prognosis

72 Pregnancy counselling: Elective Risks of pregnancy Maternal death Major complications Risk of recurrence of CHD

73 Uebing et al BMJ 2006

74 Pregnancy counselling: Elective Risks of pregnancy Maternal death Major complications Risk of recurrence of CHD Risk to the fetus: Preterm delivery/iugr/embryopathy/ intracranial bleeding

75 Uebing et al BMJ 2006

76 Pregnancy counselling: Elective Risks of pregnancy Maternal death Major complications Risk of recurrence of CHD Risk to the fetus Preterm delivery/iugr/handicap Level of monitoring and hospitalisation

77 Level of Care Required: Where? Low risk patients Locally Consultation once at ACHD & Family Planning Clinic to clarify issues/make recommendations Intermediate or high risk patients Specialized Joint Cardiac Obstetric Clinic High-risk obstetric unit with multidisciplinary care from cardiology, anesthesiology, neonatoly and nursing

78 Pregnancy counselling: Elective Risks of pregnancy Maternal death Major complications Risk of recurrence of CHD Risk to fetus Preterm delivery/iugr/handicap Level of surveillance and hospitalisation Maternal (+ paternal) life expectancy

79 Long-term Survival in Repaired CHD: Finnish Population Study Survival based on Diagnosis 84% Nieminen et al. Circulation 2001

80 Pregnancy counselling: Elective Risks of pregnancy Maternal death Major complications Risk of recurrence of CHD Risk to fetus Preterm delivery/iugr/handicap Level of surveillance and hospitalisation Maternal (+ paternal) life expectancy Contraception and other medication

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85 Risk Stratification: CHD lesion specific Left-to-righ shunts Coarctation of the aorta (reported mortality* up to 2%) Pulmonary stenosis and tetralogy of Fallot Left heart obstructive lesions (reported mortality 1-5%) Cyanotic heart disease Fontan circulation Systemic right ventricle (reported mortality 4%) Marfan syndrome Eisenmenger physiology or PAH (mortality 30-50%)

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87 Pregnancy: Repaired Tetralogy Overall risk low (<1% of major complications, incl. death) Mild to moderate PS or PR tolerated well (provided RV function is maintained) Severe PS may precipitate right heart failure, arrhythmia and sudden cardiac death: PR and or PS should be dealt with before pregnancy Associated lesions may present additional risks (residual VSD, AR with aortic dilatation need to be assessed) Infants are often SFD, low cardiac output (PR) Di George syndrome is present in 10-15% of tetralogy pts (genetic counselling) Recurrence risk with 22qD 50% + other morbidity

88 Pulmonary Regurgitation & Pregnancy: Adverse long-term effect on RV? Tetralogy of Fallot 4 p<0.02* Baseline Follow-up Right Ventricular Size p<0.02* Pregnancy Group 0 Baseline Follow-up Baseline Follow-up Control Group Uebing et al, IJC 2008

89 Hazards: Arrhythmia/Anticoagulation Treat underlying cause/s of arrhythmia Is anticoagulation necessary? Use drugs with safety record Avoid multi-drug regimes Always consider risk-benefit ratios DC cardioversion is safe

90 Anticoagulation: Prosthetic Heart Valves Avoid metalic prostheses in young women Conflict of interest between mother vs fetus Warfarin: embryopathy + intracranial bleeding LMWH with anti 10a levels + aspirin Uebing et al BMJ 2006

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94 Conclusions: Heart Disease & Pregnancy Pregnancy is feasible for most patients with Heart Disease Relatively risky endeavor compared to healthy women Aim should be to assess risks early and support patients on their desire/decision to have a family Cardiac pregnancy counseling should be outpatient based and not crisis management: Part of routine counseling of these patients and should include advice on contraception Risk stratification regarding pregnancy and HD relates to: Functional status of the patient and is Lesion specific

95 Conclusions: Heart Disease & Pregnancy Address target haemodynamic lesions/arrhythmia problems Review medication/other needs Develop and disseminate a management plan Modify labor/delivery Minimize cardiac work Monitor carefully during the peripartum Expect a good outcome Ensure appropriate cardiac follow-up

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