Clinical Case #4
Carmine Dario Vizza
Pulmonary Hypertension Unit
Dept. Cardiovascular and Respiratory Sciences Sapienza University of Rome
Director Prof. Francesco Fedele
Clinical history
SC, white female, 62-year old
- 1996: atrial fibrillation
- 2003: diabetes mellitus type II
- 2009: progressive dyspnoea and reduction of effort tolerance
- April 2010: admission for congestive heart failure; echo-Doppler estimated PAP 85 mmHg
- May 2010: patient referred to our center
Clinical Status at the Admission (May 2010)
- Dyspnoea for moderate efforts (WHO functional class III)
- Mild asthenia
- No cyanosis
- No pulmonary rales
- MIld peripheral oedema, mild epatomegaly
- Cardiac auscultation: reinforced II heart sound; olosystolic murmur (intensity 2/6)
SAP: 110/65 mmHg, HR: 90 b/min, SpO2: 93%
Electrocardiogram
- Diagnostic Work-up
- Lung Function Test (% predicted)
- FVC 78
- FEV1 80
- TLC 77
- DLCO 72
- Angio-TC: no pulmonary thromboembolism
- Autoanticorpal Screening: negative
Ecocardiography
- Right heart dilatation, with RV freewall hypokynesis (TAPSE: 12 mm)
- Mild Paradoxical movement of the IVS
- Normal left ventricular systolic function (EF 50%)
- Moderate tricuspidal regurgitation (RV-RA gradient 65 mmHg)
- Mild pericardial effusion
NYHA III
6MWT: 325 m
Baseline | NO | |
---|---|---|
Pressure (mmHg) | ||
Rap | 12 | 10 |
Pap | 34 | 24 |
PcWP | 14 | 13 |
CI (l/min/m2) | 2.6 | 2.7 |
PVT tot (WU) | 6.8 | 4.5 |
PVR art (WU) | 4 | 2.2 |
Which Diagnosis?
- Precapillary pulmonary hypertension
- No CTEPH
- No Lung Disease
- Normal LV systolic function
PAH!
but
Atrial fibrillation (usually a sign of reduced LV compliance)
Fluid Challenge !
SC, white female, 62-year old
NYHA III
6MWT: 325 m
Baseline | NO | Fluid Challenge (500 ml in 10 min) |
|
---|---|---|---|
Pressure (mmHg) | |||
Rap | 12 | 10 | 14 |
Pap | 34 | 24 | 39 |
PcWP | 14 | 13 | 18 |
CI (l/min/m2) | 2.6 | 2.7 | 2.9 |
PTR (WU) | 6.8 | 4.5 | 7.6 |
PVR (WU) | 4 | 2.2 | 4.1 |
Elevation of Pcwp!
Which Diagnosis?
Reactive (Out-of-Proportion) Pulmonary Hypertension in Group II patient
Which Therapy?
- Reactive (Out-of-Proportion, with a significant precapillary involvement) pulmonary hypertension in Group II patient
- Vasoreactivity → Calcium Channel Blocker
- Atrial Fibrillation → non-dihydroprydine Ca Channel Blocker
- Systemic Congestion → diuretics
Diltiazem 120 mg b.i.d.
Furosemide 25 mg b.i.d
Spironolactone 25 mg q.i.d
NO | ||
---|---|---|
mPAP mmHg | 34 | 24 |
CI l/min/m2 | 2.6 | 2.7 |
PVRart WU | 4 | 2.2 |
Ecocardiography 2/11
- Mild Right heart dilatation, normal TAPSE: 20 mm
- Normal left ventricular systolic function (EF 50%)
- Moderate tricuspidal regurgitation (RV-RA gradient 45 mmHg)
- No pericardial effusion
NO | ||
---|---|---|
mPAP mmHg | 34 | 24 |
CI l/min/m2 | 2.6 | 2.7 |
PVRart WU | 4 | 2.2 |
Electrocardiogram
May 2010
April 2011
How to proceed into the diagnostic work-up
Chest Pain
- Common symptom in PH
- Breathlessness
- Fatigue
- Weakness
- Syncope
- Angina
- Abdominal distension
Patient 62-year old with CV risck factor (diabetes)
But... and Indication for coronary angiography
Coronarography
Coronary angioplasty with implantation of 2 drug-eluting overlapping stents in the anterior descendent artery and in the circumflex artery
|
NYHA III 6MWT: 325 m |
NYHA II 6MWT: 470 m |
|
---|---|---|---|
Baseline | NO | Diltiazem 120 mg bid 11 months | |
Pressure (mmHg) | |||
Rap | 12 | 10 | 5 |
Pap | 34 | 24 | 24 |
PcWP | 14 | 13 | 11 |
CI (l/min/m2) | 2.6 | 2.7 | 3.1 |
PVR tot (WU) | 6.8 | 4.5 | 4.3 |
PVR art (WU) | 4 | 2.2 | 2.3 |
Conclusion
- Normal Pcwp does not always mean Precapillary PH
- The presence of clues of LV disease (AF, Diastolic Dysfunction at Echo-Doppler) should be an indication for fluid challenge
- Chest pain is associated with PH, but if patient has CV risk factors and ECG abnormalities (V4-V6) esclude coronary artery disease