Looking Through the Heart of Pulmonary Vascular Disease

PVRI Member Authors: Sachindra Joshi

“Sometimes the heart sees what is invisible to the eye” - as said by H. Jackson Brown, Jr. an american author best known for his inspirational book Life’s Little Instruction Book. In the context of pulmonary vascular diseases (PVDs) and the treatment thereof, I believe our heart can see changes in our physiology that are invisible to the eye.


Heart and lungs are intricately interrelated. Subtle change in the pulmonary circulation is detected by the patient’s heart in the pulmonary vascular disease pulmonary arterial hypertension (PAH). Increasing pulmonary arterial resistance and progressing pulmonary arteriopathy is sensed by the heart. As the resistance in pulmonary arteries increases due to progressing arteriopathy, the right ventricle is exposed to pressure overload. The right heart adapts to the increased pressure overload by right ventricular dilatation, followed by right ventricular hypertrophy. As a biomedical scientist working in pulmonary hypertension (PH) research, I have witnessed the heart of a mammal adapting to deteriorating lung pathology and increasing resistance to blood flow in the lungs by undergoing anatomical as well as molecular remodeling. Most interestingly, in pulmonary hypertension patients who have undergone a successfullung transplant, the hypertrophied heart reverts back to its original state as a normal heart. This remarkable plasticity of the heart tells us that heart can see what is at first invisible to the eyes.

In essence, despite the progressive deterioration of the lung vasculature, the heart fights until the very end to keep the patient alive. Similarly, the heart of a family audaciously adapts to the changes made to their day to day activities. For a family, pulmonary hypertension is a life-changing experience that brings many difficult challenges and changes. The emotional toll of PH is significant, and when a loved one is diagnosed with the disease, the heart of the family undergoes a lot of emotional transition, be it as a parent, a partner, a child or a friend. Apart from adapting to ever changing daily routines, the ‘family heart’ is increasingly dedicated to helping in any way, including participation in pulmonary hypertension research and awareness programs.

The heart of a clinician and a scientist is no different from a family’s heart. Clinicians work tirelessly to best manage the failing heart. They do intensive research and collaborate with various medical centers around the globe to come up with the best alternative medicine for the disease. Meanwhile, scientists are working tirelessly around the clock to solve the mystery behind the pathology of the disease and find new and alternative therapeutic targets, so that in future, pulmonary hypertension can be detected in its early stage, disease-progression may be prevented, and one day, even cured. PVRI Chronicle embraces the hearts of patients, family, clinicians and scientists in pulmonary vascular diseases, by providing a dedicated platform for news, discussion, science and medicine from all perspectives. In PVRI Chronicle, the Pulmonary Vascular Research Institute seizes the opportunity of bringing global expertise in the field of PVDs to find new solutions for improving the lives of patients, family, clinicians and scientists. Through dedicated sections such as PVRI news and activities, Journal Club, Art Club, Learner’s Corner and Patient’s Corner, PVRI Chronicle aims for widespread pulmonary vascular disease awareness.

In this issue, we have presented social and scientific news from the 8th PVRI AGM and 7th Scientific Workshops and Debates, Bad Nauheim, Germany in January; the PVRI American Thoracic Society2014 Get Together, San Diego, CA in May; and the 7th joint Saudi Association for Pulmonary Hypertension/ PVRI Conference in Muscat, Oman, April 2014. The perspective from a clinician and researcher is represented in an interview with Prof. Ghofrani, who speaks about his career path and offers advice and experience for the younger generation of PVD scientists. For the Journal club section, we have selected three interactive discussions covering a wide range of topics, from molecular links between pulmonary hypertension and obesity to the important question on exercise, and whether its benefits outweigh the negatives for pulmonary vascular disease patients. Likewise, in the Learner’s Corner, we present a historical perspective on enthothelial glycocalyx & the revised Starling principle, a review article on pulmonary hypertension in pregnancy and its prognostic implications, and a Did You Know article on Pulmonary Hypertension & HIV. The Patient’s Corner holds a Case report on PH as a function of carcinoid heart disease, as well as two perspectives, one on exercise and pulmonary hypertension and the other on the prevalence, causes, clinical characteristics and diagnostic challenges associated with pulmonary hypertension (PH) in African and African-Americans.

Our seven pound heart puts all its effort in the fight against the increasing pressure overload due to increased pulmonary arterial resistance in pulmonary hypertension. Similarly, if each individual in our PVD community puts a little effort in communicating their science, PVD issues, news and scientific debates, these informative works collectively will help improve the heart of the pulmonary vascular disease community. Therefore, I urge you on behalf of our editorial board to send articles for the forthcoming issues of PVRI Chronicle and help in spreading pulmonary vascular disease awareness worldwide.

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PVRI Chronicle Vol 1: Issue 2 cover image

December 2014

PVRI Chronicle Vol 1: Issue 2

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