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