Cardiovascular Disease |

Unique Coexistence of Severe Primary Pulmonary Hypertension and Marfanoid Habitus in a Young Male - An Interesting and Rare Case Report FREE TO VIEW

Ram K. Chopra, MD; Sanesh Garde, DTCD; Chintan Patel, MD; Dhiraj Jain, MD; Omkar Kajale, DTCD; Rahul Jalan, MD
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Ruby Hall Clinic, Pune, India

Chest. 2014;145(3_MeetingAbstracts):64A. doi:10.1378/chest.1832842
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SESSION TITLE: Cardiovascular Case Report Posters I

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

PURPOSE: Primary pulmonary hypertension is severe and progressive disease with incidence of 1-2 cases / million / annum. Without treatment, survival is 2-3 years. Only 10% survive for 10 years. Co-existence of PPH with Marfanoid Habitus has not been reported till date in any literature or case study.Large series of 120 PPH patients from Mayo clinic by Fuster et al showed thrombotic mechanism by microemboli and plexogenic arteriopathy due to hyper reactive pulmonary arteries by open lung biopsies.

METHODS: A 32 yr non smoker male presented with progressive dyspnea, chest pain and dry cough. He denied history of fever, joint pains, skin rash or any drug intake. Patient had consulted physicians for last 6 months without relief. Family history was noncontributory & no sudden deaths. On examination he was tall, thin with arm span greater than the height, arachnodactyly. He had tachypnea, BP- 100/60 mmhg & oxygen saturation- 82%. Auscultation- loud P2, pansystolic murmur at lower sternal border. Remaining systems were normal. ECG -right heart strain pattern. Routine blood investigations were normal.Collagen profile and serology was negative.Chest radiography -enlarged left pulmonary conus. DLCO was decreased . TEE showed PASP of 165mmhg, severe TR, RA and RV dilatation besides other abnormal findings.No left to right shunt and left heart was normal. HRCT thorax was normal. CT pulmonary angiography revealed PA diameter of 3.7cm and no evidence of pulmonary embolism. Patient was not willing for cardiac catheterization.

RESULTS: Careful examination and investigations led us to diagnose this rare & interesting case of Primary Pulmonary Hypertension with combination of Marfanoid Habitus. Treated with drugs as per protocol, O2 and life style modifications with improvement.

CONCLUSIONS: One of the rare causes of dyspnea is PH & PPH further itself is a very rare entity and due to its greivous nature and high mortality, active consideration, special investigations and early treatment should be initiated when other commom causes are excluded. Further research into understanding the pathogenesis and treatment of PPH is imperative to decrease the high mortality associated with the disease.

CLINICAL IMPLICATIONS: Hypoxia is a potent exacerbating factor in PPH hence strenous activity should be avoided. Treatment includes oxygen supplementation, anti-coagulants, diuretics, calcium channel blockers, prostanoids, endothelin receptor antagonist, phosphodiesterase-5 inhibitors. Influenza and pneumococcal vaccine is strongly recommended.

DISCLOSURE: The following authors have nothing to disclose: Ram k Chopra, Sanesh Garde, Chintan Patel, Dhiraj Jain, Omkar Kajale, Rahul Jalan

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