SHA24/004005

ASD In Adults PAH Management updates Dr. Ahmed Samman Consultant Adult Cardiologist Head Adult Cardiology Director Non-invasive Cardiology Lab Adult Congenital Heart Disease King Abdullah Medical City in Holy Capital Feb-13-2013

ASD and PAH In Adults Presentations in adults (secondum ASD cases) Eisenmenger syndrome Management Medical Treat and repair ?????

ASD

Clinical classification of congenital, systemic-topulmonary shunts associated with pulmonary arterial hypertension

Eisenmenger syndrome Eisenmenger syndrome (ES) represents the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart defects

Eisenmenger syndrome The triad of : systemic-to-pulmonary communication, pulmonary vascular disease and cyanosis

Natural history the likelihood of developing ES depends on size and localisation of the intracardiac defect: - VSD: 3% of pts. with VSD <1.5cm 50% of pts. with VSD >1.5 cm - ASD: 10% of large ASD (higher in ASD I, AVSD) - PDA: 16% of moderate or large PDA - truncus arteriosus: all most all pts.

PDA, VSD present earlier (80% during infancy) than ASD (90% during adulthood).

Case-1 Age O2 % Size/Rim PAP/ PVR

Rhythm RV/LV

Comorbidity Management

Case-2

Case-3

Case-4

ASD-3

ASD-3

Case-1

Case-3

Age y O2

25 85-90%

Size/Rim

22 mm good

PAP/ PVR

100 ↑

Rhythm

NSR

RV/ LV

Severe/ Normal

comorbidity

↓ RV Management

Treat and ± ?repair

Case-5

ASD In Adults: Clinical Management Updates

ASD In Adults Clinical updates -Impact of -Morphology of ASD -Age -PAH -Arrhythmia -HTN and CAD -RV and LV dysfunction

Imaging updates -Echo -MRI Management updates -Devise vs. surgical Outcome of ASD closure - RV and LV remodeling - MVO2 max

Hemodynamic parameters according to age group in 505 adult patients with an atrial septal defect that was unrepaired at baseline.

PAH and ASD Incidence of 6-15% in the ASD population

PAH and ASD

PAH and ASD hypertension related to the shunt, but they may have pressure elevation, as a result of the development of : pulmonary parenchymal disease Thromboembolic disease left-sided heart dysfunction obstructive sleep apnea

It would be fair to say that the overall risk of and specific risk factors for developing pulmonary vascular disease with an ASD remain unknown.

PAH and ASD

PAH and ASD Retrospective 54 patients with mean PAP 57 ± 11 moderate (n=34) or severe PAH (n=20) Early F/Up 2.3 ±1.2 months Late F/Up 31 ± 15 months

ASD

PAH and ASD Early improvements in RVSP are seen in patients with moderate or severe PAH undergoing transcatheter ASD closure.

Continued improvement in RVSP occurs in late follow-up.

Despite decreases in the mean RVSP in late follow-up, many patients do not have complete normalisation of pressures.(15%)

PAH and ASD

Treat-and-Repair strategy

ASD The magnitude of and direction of flow through an ASD depend on -the size of the defect and -the relative diastolic filling properties of the left and right ventricles.

RV-diastolic dysfunction

LV-diastolic dysfunction

Severe PHTN

Systemic HTN-?MS

ASD

R- L shunt

Popoff for the RV

L- R shunt Popoff for the LV

ASD and Cardiac Imaging

Multimodality Imaging in ASD Technique

Indication

Information

TT/TE ECHO

• •

• • • •

•

Diagnosis of ASD Differential Diagnosis Evaluation of antomy

• •

3D Echo

Difficult ASD sizing /morphology

Type of ASD: secundum , primum, sinus defect Measurements of ASD Evaluation of ASD rims Visualization of Chiari network, Eustachian valve, embryonal remnants of early septation Evaluation of previous ASD repair PAP

Evaluation of rims and ASD size in difficult cases

Multimodality Imaging in ASD Technique Computed tomography

Indication •

•

MRI

• •

Information

Diagnosis of ASD + anomalous venous return Coronary angiography in mid age patients with ASD

• • •

Visualization of anomalous venous return Visualization of coronary arteries before any ASD interventions Pulmonary Thromboembolism

Diagnosis of ASD Association with other CHDs

• • • • • •

Type of ASD: secundum , primum, sinus defect Measurements of ASD Evaluation of ASD rims Association with venous return QP/QS Evaluation of associated CHDs

Vasoreactivity response Dose PVR fall when giving a pulmonary VD? Purpose IPHT: Therapy (Ca channel Blocker)?

CHD (shunt lesion):Is intervention safe?

In CHD : intervention is device or repair ?

Vasoreactivity response PAP/ transpulmonary gradient PVR indexed PVR/SVR ratio Qp/Qs An acute reduction of the mean pulmonary arterial pressure of >10 mm Hg with a resultant mean pulmonary arterial pressure of 40mmHg or less without a fall in cardiac output

ASD Management

CANADIAN CARDIOVASCULAR SOCIETY 2009 CONSENSUS CONFERENCE UPDATE ON THE GUIDELINES FOR THE MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE Presentation at Annual CCS Meeting in Edmonton 2009

Presentation at Annual CCS Meeting in Edmonton 2009

PART I ASD VSD AVSD PDA Dylan A. Taylor MD FRCPC FACC Director, Northern Alberta Adult Congenital Heart Clinic University of Alberta Mazankowski Alberta Heart Institute Edmonton, Alberta, Canada

Presentation at Annual CCS Meeting in Edmonton 2009

Atrial Septal Defect – Class III  If PAH is present and there is irreversible PAH, the

ASD should not be closed. Such patients should receive care from a specialist with expertise in PAH. (Level: C)  pulmonary artery pressure (PAP) > 2/3 systemic arterial blood pressure (SABP)  pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance

Presentation at Annual CCS Meeting in Edmonton 2009

AT-Management

Bosentan therapy

54 patients 16 weeks

Combined therapy Bosentanâ&#x20AC;&#x201C;sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology Michele etal. IJC March-2012

Methods Thirty-two patients with CHD-related PAH (14 male, mean age 37.1Âą13.7years) treated with oral bosentan underwent right heart catheterization (RHC) for clinical worsening.

After RHC, all patients received oral sildenafil 20mg thrice daily in addition to bosentan.

Clinical status, resting transcutaneous oxygen saturation (SpO2), 6-minute walk test (6MWT), serology and RHC were assessed at baseline (before add-on sildenafil) and after 6months of combination therapy.

Conclusion Addition of sildenafil in adult patients with CHD-related PAH and Eisenmenger syndrome after oral bosentan therapy failure is safe and well tolerated at 6month follow-up, resulting in a significant improvement in clinical status, effort SpO2, exercise tolerance and haemodynamics.

Treat-and-Repair strategy

Thank you