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

 

Casi clinici

• Clinical Case #4