1. Clare O’Donnell Paediatric/Adult Congenital Cardiologist
Tips for Evaluation of Pulmonary Hypertension in the Adult with Congenital Heart Disease
Clare O’Donnell
Paediatric/Adult Congenital Cardiologist
Green Lane Paediatric and Congenital Cardiac Service

4. Questions
Functional status Severity Reversibility Prognosis Treatment and Operability

5. Eisenmenger syndrome
32 yo man Non restrictive VSD Cyanosis Dyspnea Reasonably active until 3 years before his death Developed progressive CHF, died of hemoptysis
Eisenmenger V. Z Klin Med. 1897; 32

7. Nice 2013 PAH/CHD Eisenmenger syndrome Left to right shunts
Correctable
Noncorrectable
PAH with coincidental CHD
Post operative PAH
JACC Vol 62, No.25, Suppl D, 2013

8. Tests – traditional and evolving
Anatomy – Echo (TTE, TOE), CT Hemodynamics Genetic testing Echo parameters of RV function TAPSE, RV strain, Fractional area change MRI
Gatzoulis group – high rates of LGE and poorer outcome correlates with impaired RV function, impaired LV function and particularly biventricular impairment Circ CV imaging 2015

9. Impaired Right, Left, or Biventricular Function and Resting Oxygen Saturation Are Associated With Mortality in Eisenmenger Syndrome A Clinical and Cardiovascular Magnetic Resonance Study
Jensen et al, Circulation CV imaging 2015

10. Functional status
NYHA/Ability Index QoL scores - Camphor and others Exercise testing 6 Minute walk CP Exercise testing

11. Other parameters
Eisenmenger group – predictors of death Age Pretricuspid shunt Oxygen saturation at rest Presence of sinus rhythm Presence of pericardial effusion Kempney et al Circulation April 2017
Multicentre, approx 1100 eisenmenger patients – predictors of death of group with mean age early 30s in mulitvariate analysis at 3 years -

12. Cardiac Cath – what do we want to know?
Understand anatomy
Venous occlusion
Phobias
Developmental issues

14. Need to clearly understand the anatomy and be systematic !?
Pulmonary venous desaturation
Systemic desaturation CI
PVR(I)
Qp:Qs
Rp:Rs
2 (or more) sources of Pulmonary blood flow ?
Bilateral SVCs?
IVC interruption
Anomalous pulmonary venous drainage?
Low Cardiac output

15. Complex Pulmonary Supply and Drainage

18. Fontan circulation Non pulsatile flow
Loading conditions eg dehydration
Sedation/Ventilation
End expiratory pressures

19. saturation provides a good prediction of surgical outcome.’
‘In conclusion
(1) Atrial septal defect with high total pulmonary resistance is uncommon and predominates in adult female patients.
(2) Total pulmonary resistance (or pulmonary arteriolar resistance) is the best predictor
of surgical outcome. In patients with total pulmonary resistance less than 15 U/m2, surgical treatment is advised.
(3) In patients with borderline total pulmonary resistance, the systemic arterial oxygen
saturation provides a good prediction of surgical outcome.’
Circulation 76, No. 5, , 1987.
Saturations over 92%

20. Suitable for closure ?

23. Other contributors Obesity/OSA Diastolic dysfunction
Abnormal pulmonary function
Thrombus
Genetics

24. Importance of Follow up