01 March 2017 by Roxana Sulica

Distinctive clinical and hemodynamic characteristics of IPAH patients of Indian descent from Guyana

Introduction 

Little is known about the ethnic disparities among pulmonary hypertension patients. We present a case series of Guyanese patients of Indian descent with severe pulmonary hypertension, high prevalence of right ventricular hypertrophy, and high mortality despite intensive PAH therapy.  These characteristics are particularly expressed in patients diagnosed with IPAH prior to the age of 45. 

Methods

We have retrospectively reviewed the charts of consecutive IPAH patients followed in our program from 2000-2016 and we have identified nine patients of Indian descent born in Guyana.  We have analyzed clinical (WHO functional class, 6-minute walk distance), echocardiographic and hemodynamic data on presentation and we recorded treatment patterns and outcomes. We are presenting data for the entire group and for 7/9 patients diagnosed prior to the age of 45.

Results

There were 7/9 women, with mean age at diagnosis of 40.2 years; mean duration of follow-up was 6.4 years.  Baseline mean 6-minute walk distance for the group was 336 m (324 m for patients diagnosed prior to the age of 45).  Upon presentation, patients diagnosed prior to the age of 45 had WHO functional class III or IV symptoms, while older patients presented in WHO functional class II.  In patients diagnosed prior to the age of 45, right ventricular hypertrophy was present in 6/7 and pericardial effusions in 5/7; they all had severe dilatation of the right atrium and ventricle and severe RV dysfunction, and paradoxical septal motion.  Echocardiograms in the 2/9 patients diagnosed later than 45 years of age lacked all stigmata of severe RV dysfunction.  All patients received three-drug combination (endothelin receptor blocker, NO pathway drug, and prostacyclin pathway agents), with 7/9 patients requiring parenteral prostacyclins.  Five out of nine patients died (55% mortality at 6 years), with four deaths due to right heart failure (including one death while awaiting transplantation) and one death due to Stenotrophomonas maltophilia central line sepsis.  All deaths were recorded in the younger patient group and all of them were receiving parenteral prostacyclin at the moment of death.  Hemodynamic data is presented in table 1.  

 

All group (n=9)

Diagnosed <45 yo (n=7)

RAP (mmHg)

14

15

Mean PAP (mmHg)

72

79

CI (L/min/m2)

1.9

2.0

PVR (wood units)

20

22

PA saturation (%)

54

51

  • RAP=right atrial pressure
  • PAP=pulmonary artery pressure
  • CI=cardiac index
  • PVR=pulmonary vascular resistance
  • PA=pulmonary artery. 

Discussion

In this case series of Guyanese patients we have noted a high prevalence of severe pulmonary hypertension with poor response to therapy and poor outcomes.  It is unclear if this specific phenotype has either a genetic or environmental explanation, but further studies of this patient population may offer insights in the pathophysiology of PAH.

Key Contributors

Swathi Sangli MD and Roxana Sulica MD


Comments (0)

Our research platform is the world.

Through worldwide collaboration, we can begin to answer the question of a global disease.

Join the PVRI
standard-example-image.jpg