Haemodynamic spectrum in heritable pulmonary arterial hypertension: a continuum from pre-capillary to combined pulmonary hypertension—case series

Figures

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  Figure 1. Resting right heart catheterization tracings during follow-up of Case 1 at time of hospital admission. (A) Mean right atrial pressure 14 mmHg. (B) Pulmonary artery pressures 68/38/49 mmHg. (C) Mean pulmonary arterial wedge pressure 18 mmHg. Note the significant respiratory variations due to obesity. PAp, pulmonary artery pressures; PAWP, pulmonary arterial wedge pressure; RAp, right atrial pressure.

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  Figure 2. Resting right heart catheterization tracings during follow-up of Case 2 at time of hospital admission. (A) Mean right atrial pressure 18 mmHg. (B) Pulmonary artery pressures 68/32/39 mmHg. (C) Mean pulmonary arterial wedge pressure 23 mmHg. PAp, pulmonary artery pressures; PAWP, pulmonary arterial wedge pressure; RAp, right atrial pressure.

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  Figure 3. Transthoracic echocardiography during follow-up of Case 2 at time of hospital admission. (A) Apical four chamber view depicting a severely dilated right ventricle with the following dimensions: end-diastolic right ventricular basal diameter 52 mm, end-diastolic right ventricular mid diameter 55 mm, end-diastolic right ventricular longitudinal diameter 85 mm, right ventricular end-diastolic area 41.5 cm2. Right ventricular systolic dysfunction was severely reduced (fractional area change 19%, tricuspid annular plane systolic excursion 12 mm with severe tricuspid regurgitation, pulsed Doppler S wave of the tricuspid annulus 6.9 cm/s). Severe right atrial dilation (33 cm2). (B) Right ventricular-focused apical four chamber view showing severe functional tricuspid regurgitation. (C) Apical four chamber view depicting moderate mitral regurgitation due to anterior leaflet prolapse. (D) Moderate functional pulmonary regurgitation allows for estimation of a mean pulmonary artery pressure of 45 mmHg. (E) Dilated inferior vena cava of 24 mm without respiratory variation, estimated right atrial pressure of 15 mmHg. (F) Mitral inflow shows pseudonormal left ventricular filling pattern…

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  Figure 4. Transthoracic echocardiography of Case 3 at screening for pulmonary hypertension. (A) Systolic peak tricuspid regurgitation velocity 3.0 m/s. (B) Shortened right ventricular outflow tract acceleration time of pulmonary ejection of 70 ms without evident mid-systolic notch. (C) Apical four chamber view depicting normal right ventricular, right atrial, and left ventricular size and mild left atrial dilation (35 mL/m2). (D and E) Mitral inflow shows pseudonormal left ventricular filling pattern consistent with Grade 2 diastolic dysfunction (elevated left ventricular filling pressures). Mitral E-wave was 82 cm/s; mitral A-wave was 70 cm/s. Valsalva manoeuvre increased A-wave velocity. (F) Reduced lateral mitral e′-wave in tissue Doppler imaging of 8.8 cm/s. Average E/e′ ratio was 11.

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  Figure 5. Mean pulmonary artery pressure and mean pulmonary arterial wedge pressure/cardiac output slopes at peak exercise of Case 3 during screening for pulmonary hypertension. The mean pulmonary artery pressure/cardiac output slope was 3.7 mmHg/L/min and the mean pulmonary arterial wedge pressure/cardiac output slope was 3.8 mmHg/L/min. CO, cardiac output; mPAP, mean pulmonary artery pressure; PAWP, mean pulmonary arterial wedge pressure.

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  Figure 6. Pulmonary angiography images of the pulmonary arteriovenous fistulae during embolization. (A) Pulmonary arteriovenous fistula in the basal region of de middle lobe (arrow). (B) Pulmonary arteriovenous fistula in the right lower lobe (arrows). (C) Status post-embolization of the fistula in the basal region of the middle lobe with a 6 mm large Amplatzer device (orange arrow) and of the fistula in the right lower lobe with four 5 mm coils (yellow arrow). Note that three smaller fistulae could technically not be embolized.

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  Figure 7. Wasserman curves of the cardiopulmonary exercise test of Case 4 during screening for pulmonary hypertension. Exercise was performed on upright cycle ergometer. The test was maximal (respiratory exchange ratio at peak exercise 1.12, Curve 8). Functional capacity was mildly reduced [peak oxygen consumption 16.5 mL/Kg/min (69% of predicted), Curve 3]. There was moderate ventilatory inefficiency: ventilatory equivalent of carbon dioxide at first ventilatory threshold was 38 (138% of predicted) and ventilatory equivalent of oxygen at peak exercise was 46 (Curve 6). Ventilation/carbon dioxide production slope was 34.8 (Curve 4). End-tidal carbon dioxide partial pressure at rest was 24 mmHg, at first ventilatory threshold 30 mmHg, at peak exercise 28 mmHg (Curve 9). AT, first ventilatory threshold; Bf, breathing frequency; BR, breathing reserve; EqCO2, ventilatory equivalent of carbon dioxide; EqO2, ventilatory equivalent of oxygen; HR, heart rate; PETCO2, end-tidal carbon dioxide partial pressure; PETO2, end-tidal oxygen partial pressure; RCP, second ventilatory threshold; RER, respiratory exchange ratio; VE, ventilation; VE/VCO2, ventilation/carbon dioxide production; VO2, oxygen…

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  Figure 8. Resting and exercise right heart catheterization tracings of Case 4 during screening for pulmonary hypertension. (A) Resting pulmonary artery pressures 33/15/22 mmHg. (B) Resting mean pulmonary arterial wedge pressure 13 mmHg. (C) Pulmonary artery pressures 56/30/41 mmHg at peak exercise. (D) Mean pulmonary arterial wedge pressure at peak exercise 34 mmHg. PAp, pulmonary artery pressures; PAWP, pulmonary arterial wedge pressure; pEx, peak exercise.