Unilateral left Pulmonary Artery Agenesis with Pulmonary Hypertension: Unusual First Presentation.

Introduction: Unilateral absence of pulmonary artery is arare congenital disorder. We present here the case of Unilateralleft pulmonary artery agenesis with pulmonary hypertension.Case Report: A 33-year young male patient presented toour tertiary care hospital in the out-patient department withcomplaints of shortness of breath on exertion for 7 months andbilateral feet swelling for 6 months. Routine investigationswere within normal limits except 2 dimensional ECHOshowed right atrium and right ventricle dilatation with severepulmonary artery hypertension and 60% left ventricularejection fraction. Patients with isolated right pulmonary arteryagenesis commonly survive into adulthood with minimal orno symptoms, which makes their identification challenging.But our patient had left pulmonary artery agenesis whichhas significant association of life threatening cardiovascularmalformations with left-sided UAPA and surgical repair isoften required during the first year of life. None of which ispresent in our patient which is unsusual.Conclusion: Clinicians should be aware that recurrentrespiratory infections may be presenting feature ofUAPA. Initial investigation is usually a chest radiograph.Echocardiography is required for evaluation of possiblepulmonary hypertension. Confirmation of the diagnosis andanatomic details can be discerned by CT scanning and MRI.Angiography is reserved for patients requiring embolizationor revascularization surgery.Present case demonstrates a rare presentation of UAPA asright heart failure in first visit.

Hyperhomocystetenemia, MTHFR Mutation Presenting as Unprovoked Pulmonary Embolism.

Introduction: For MTHFR as with homocysteine testing,no official guidelines exist as to who should be tested.Homozygosity for the MTHFR C677T mutation has beenassociated with an increase in blood clotting together withplasma homocysteine increase and DVT occurrence risk.Case report: A 28 year young male patient presented withcomplaints of sudden onset breathlessness for 5 days. Theepisodes of breathlessness were associated with diffuseanterior chest pain. There was no history of leg pain, cough,sputum, hemoptysis, fever. No history of prior hospitalization,trauma, surgery and immobilization could be elicited from thepatient. He was a non smoker with no other comorbidities.On presentation his pulse rate was 120 per minute, respiratoryrate was 22 per minute, blood pressure 146/92 mm Hg,temperature 98.8 ° F, SpO2 of 94% at room air. His generalphysical examination was unremarkable.Conclusion: Although it has been observed that elevatedhomocysteine levels are a common finding in patientswith cardiovascular disease and thrombosis, its role in itspathogenesis is still under evaluation. Homozygosity forthe MTHFR C677T mutation has been associated withincreased homocysteine levels. Testing for this mutation is animportant parameter in thrombophilia workup of patients withunprovoked VTE.

Assessment of the correlates of respiratory morbidity related quality of life, using St George Respiratory Questionnaire (SGRQ) among male COPD patients

Background: COPD is ranked eighth among the top 20 conditions causing disability globally. Assessment in subjective areas such as dyspnea and HRQL provides complementary information to physiologic measurements. Lower Health-Related Quality of Life has been associated with mortality and morbidity in COPD.Methods: The study was conducted at the Department of Respiratory Medicine and at Vallabhbhai Patel Chest Institute and the associated, Vishwanathan Chest Hospital, University of Delhi. Between September 2012 to August 2013. We conducted present study on 40 male COPD subjects aged more than 45 years, divided into 4 groups based on CT phenotype as normal, Airway Dominant (AD), Emphysema Dominant (ED) and mixed types. We compared the St. George Respiratory Questionnaire scores, 6 Minutes’ walk Distance scores, Clinical parameters, Spirometry indices across these phenotypes. Results: The mean SGRQ score in present study was 54.07 ± 17.24 (Range :17.3 to 84.57). The Mean 6MWD in present study was 434.58 ± 125.47 metres. The significant parameters which had correlation with SGRQ total score were Age (r=0.343, p = 0.03), 6MWD (-0.397, p = 0.011), FEV1 /FVC (0.499, p< 0.001), DLCO (-0.601, p <0.001), Low attenuation areas in CT (0.606, p< 0.001). Conclusions: 6MWD, FEV1/FVC, age, Low attenuation areas in CT, DLCO had an influence on the quality of life as measured by SGRQ scores in present study. Therapeutic approaches to improve the quality of life in COPD should take these indices into consideration.

An Unusual Cause of a Pulmonary Mass: Actinomycosis.

We present the case of a 62-year-old male with chronic obstructive pulmonary disease and poorly controlled diabetes mellitus who presented with haemoptysis. A radiograph of the chest showed a right lower parahilar opacity which on the contrast enhanced computed tomography was seen to be an irregular, spiculated mass localised to the middle lobe. Considering malignancy as the most probable diagnosis, a bronchoscopic endobronchial biopsy was performed which surprisingly established pulmonary actinomycosis as the diagnosis. The patient was successfully managed with amoxicillin and clavulanic acid and glycaemic control.