Question 2

College Answer

a)

RV size: LV size on apical 4-chamber view RV diameter

RV wall thickness

Tricuspid annular plane systolic excursion (TAPSE) or S-PRIME on apical 4-chamber view

Right ventricular systolic pressure gradient to right atrium using tricuspid regurgitation (TR) jet. Ventricular septal motion (D-shaped septum) that can indicate pressure or volume overload

Tissue Doppler and E/E’ ratios

b)

Elevated Jugular venous pressure

Right ventricular heave

Right ventricular third heart sound

Pleural effusion

Peripheral oedema Enlarged liver edge Ascites

c)

1. Pulmonary arterial hypertension (PAH)

Idiopathic PAH, Heritable-genetic disease, Drugs and toxins induced: appetite suppressants e.g. fenfluramine, Associated with systemic disease: Connective tissue diseases e.g. scleroderma, HIV infection, Porto-pulmonary hypertension

2. Pulmonary hypertension due to left heart disease

Systolic dysfunction, Diastolic dysfunction, Valvular disease: Mitral stenosis, Mitral Regurgitation, Congenital abnormalities

3. Pulmonary hypertension due to lung diseases and/or hypoxia

Chronic obstructive pulmonary disease, Interstitial lung disease, Sleep-disordered breathing, Alveolar hypoventilation disorders, Chronic exposure to high altitude

4. Chronic thromboembolic pulmonary hypertension

5. PH with unclear and/or multifactorial mechanisms

Hematological disorders: myeloproliferative disorders, Systemic disorders: sarcoidosis, vasculitis, Metabolic disorders: glycogen storage disease, Others: tumour obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

Additional Examiners’ Comments:

Some candidates provided more than four answers for parts a) and b) and it should be noted that only the first four answers are considered. Part c) in general was poorly answered and many candidates confused acute elevations in pulmonary pressure with the disease entity of pulmonary hypertension.

Discussion

a) For the RV parameters detectable with TTE, I went to the 2010 ASE Guidelines for the echocardiographic assessment of the right heart in adults.

In brief, the assessable parameters are:

• RV dimensions
• RA dimensions
• RVOT dimensions
• RV wall thickness
• IVC dimensions
• RV systolic function
  • Several parameters: RIMP, TAPSE, 2D RV FAC, 2D RV ejection fraction (EF), three-dimensional (3D) RV EF, tissue Doppler–derived tricuspid lateral annular systolic velocity (S'), and longitudinal strain and strain rate.
• RV diastolic function
  • Various parameters: E/A ratio, deceleration time, the E/e' ratio, and RA size
• Pulmonary systolic pressure/RSVP

b) Clinical signs of right heart failure are difficult to find in any one single resource.

• Features attributable to pulmonary hypertension
  • Loud P2(may be palpable)
  • Narrowly split S2
  • Tricuspid murmur
  • Diastolic murmur of pulmonary regurgitation
• Features attributable to RV hypertrophy
  • Prominent a  wave in the JVP
  • Right-sided fourth heart sound (augmented by inspiration)
  • Left parasternal heave
  • Downward subxiphoid thrust.

• Features attributable to RV dilatation and decompensated failure
  • Prominent v wave in the significantly raised JVP
  • Right-sided third  heart sound (augmented by inspiration)
  • Peripheral oedema
  • Ascites
  • Hepatomegaly (which may be pulsatile)
  • Signs of LV failure, eg. pulmonary oedema (due to out-bowing of the intraventricular septum, and LV diastolic failure resulting from this)

c)

The list used by the college answer is the updated 2013 Dana Point classification, which can be found in the Siomonneau article in Table 1.

Classification of Pulmonary Hypertension

Pulmonary Arterial Hypertension	Idiopathic Herediary or congenital Familial PAH BMPR2 mutations (a member of the transforming growth factor β signaling family) Congenital systemic-to-pulmonary shunts Eisenmenger syndrome Drug and toxin induced Definite association: aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil Possible association: cocaine, phenylpropanolamine, St Johns Wort, chemotherapy agents, SSRIs Associated with connective tissue disease Systemic sclerosis SLE Sjogren syndrome polymyositis rheumatoid arthritis Associated with HIV infection clinical, hemodynamic, and histologic characteristics similar to those seen in idiopathic PAH Portopulmonary hypertension 2% to 6% of patients with portal hypertension have PAH Pulmonary vascular resistance (PVR) is usually normal in these cases
Left heart disease	LV failure Mitral valve disease Congenital or idiopathic cardiomyopathies, eg. HOCM
Lung disease or hypoxia	Idiopathic pulmonary fibrosis (by virtue of fibrosis) Chronic hypoxia: Alveolar hypoxia as a result of lung disease, eg. COPD or pulmonary fibrosis Alveolar hypoxia due to impaired control of breathing (eg. OSA)\ Alveolar hypoxia due to residence at high altitude
Thromboembolism	obstruction of pulmonary arterial vessels by thromboemboli, tumors, or foreign bodies
Pulmonary hypertension due to unclear or multifactorial aetiologies	polycythemia vera essential thrombocythemia chronic myeloid leukemia Chronic haemolytic anaemia ​​​​​​​sickle cell disease (SCD) thalassemia hereditary spherocytosis stomatocytosis microangiopathic hemolytic anemia sarcoidosis Langerhans histiocytosis glycogen storage diseases Gaucher disease mediastinal fibrosis Schistosomiasis Embolic obstruction of pulmonary arteries by schistosoma eggs local vascular inflammation as a result of impacted schistosoma eggs

Simonneau, Gérald, et al. "Updated clinical classification of pulmonary hypertension." Journal of the American College of Cardiology 54.1s1 (2009): S43-S54.

Rudski, Lawrence G., et al. "Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography: endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography." Journal of the American Society of Echocardiography 23.7 (2010): 685-713.