01 March 2017 by Zeki Ongen

Swinging between Group 1 and Group 2 pulmonary hypertension: a case presentation

Background

Grouping of pulmonary hypertension (PH) is essential for the establishment of proper therapies. However in some clinical scenarios the differential diagnosis is challenging. This is true especially for the patients who are on the edge of combined post-capillary and pre-capillary PH (Cpc-PH) and pre-capillary PH.

Case

A 64-year-old male presented with dyspnea of FC NYHA III and swelling of legs. In his physical examination a loud P2 and a holosystolic murmur at the lower sternal border were heard. Peripheral severe edema was present. ECG revealed right ventricular hypertrophy with sinus rhythm.  In echocardiographic examination:  

RA mm

RV mm

IVS mm

LA mm

LVEF %

PASP mmHg

69

67

11

39

55

90

His serologic, rheumatologic and respiratory examinations were within normal limits except elevated proBNP. After intensive diuretic therapy he underwent an invasive evaluation. Coronary arteries were normal and his hemodynamic examination was as follows: 

RA mmHg

PA mmHg

PAWP mmHg

LVEDP mmHg

CO L/min

CI L/min/m2

PVR Wood U

12

80/30;45

12

11

4.5

2.1

6.9

He was diagnosed as IPAH and since he was not vasoreactive bosentan therapy was initiated. However, in spite of add on sildenafil, every 2-3 months he was re-hospitalized with clinical deterioration, without any significant changes in his echocardiographic and hemodynamic findings.  In the second year of his follow-up increase in LA dimensions and IVS thickness were observed. In hemodynamic examination with volume challenge PAWP increased from 14 mmHg to 22mmHg. PAH specific drugs were stopped and he is currently on HFpEF treatment without any hospitalizations for 1.5 years.

Conclusion

The need for standard tests to reveal Cpc-PH is obvious. 

Key Contributors

Burcak Kilickiran Avci1, Ersan Atahan2, Gul Ongen2, Zeki Ongen1 Istanbul University, Cerrahpasa Medical Faculty, Cardiology1 and Pulmonary Disease2 Departments


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