Abstract
Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.
Highlights
IntroductionAbout the Author: Rahul Surviving adults with an uncorrected double inlet left ventricle
About the Author: Rahul Surviving adults with an uncorrected double inlet left ventricleRegi Abraham is a internal in Dr B
DILV known as “Single Ventricle” is a congenital heart defect where both the left and the right atrium opens into the left ventricle (Compare figure 1a. of normal heart and1b. of a heart with DILV)
Summary
About the Author: Rahul Surviving adults with an uncorrected double inlet left ventricle. Available from: https://www.bhf.org.uk/publications/children-and-young-people/understanding-your-childsheart---double-inlet-ventricle) Reprinted with permission from British Heart Association. Reprinted with permission from British Heart Association tion for the same he was found to have high hemoglobin levels and has since been asked to perform regular phlebotomies if his hemoglobin crossed 16g/dl (last phlebotomy in 2015) He gives history of difficulty gaining weight as a child and history of repeated respiratory tract infections. Patient has red conjunctiva; clubbing (Grade IV); cyanosis of lips, fingers and tongue His vital showed pulse: 92 beats per min, regular rhythm, normal in volume and character; Respiratory rate: 26 breaths/min, abdomino-thoracic respiration. Investigations A review of the patient’s files the patient showed that the diagnosis of double inlet left ventricle was made at a tertiary level hospital but no therapeutic interventions were performed nor regular follow ups were made. Up to this day of writing this case report the patient has not visited a tertiary center and continues with occasional phlebotomies
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
