Diagnostic Pitfall: Mixed Connective Tissue Disease-Associated Pulmonary Hypertension Presenting With Postpartum Right-Sided Heart Failure in a 21-Year-Old-Case Report.
11 affiliations
- 1Andhra Medical College Visakhapatnam Andhra Pradesh India.
- 2Vedantaa Institute of Medical Sciences Dahanu India.
- 3Department of Pediatrics Andhra Medical College Visakhapatnam Andhra Pradesh India.
- 4NRI Medical College and General Hospital Guntur India.
- 5All India Institute of Medical Sciences Bhopal Bhopal India.
- 6ESIC MC and Hospital Chennai India.
- 7Kasturba Medical College Mangalore India.
- 8Internal Medicine Kasturba Medical College Mangalore India.
- 9Lokmanya Tilak Municipal Medical College and General Hospital Mumbai India.
- 10Shadan Institute of Medical Sciences, Teaching Hospital and Research Center Hyderabad India.
- 11Faculty of Medicine and Biomedical Sciences University of Garoua Garoua Cameroon.
Abstract
Mixed connective tissue disease is a multisystem autoimmune condition characterized by overlapping clinical and serological features related to anti-U1-RNP antibodies of at least two connective tissue diseases. It may present with myocarditis, pericarditis, pulmonary arterial hypertension, and, less commonly, heart failure. We present a 21-year-old female with bilateral foot swelling, cough, and shortness of breath 3 months after a vaginal delivery. On examination, she was hypotensive (90/70 mmHg), with an elevated jugular venous pressure, a pulmonary systolic murmur with loud P2, and a tricuspid regurgitation murmur. Bronchial breath sounds and fine crepitations were present in both lower lung fields. She was evaluated initially for suspected postpartum cardiomyopathy. Transthoracic echocardiography was suggestive of a probable moderate to severe PAH. High-resolution computed tomography showed bilateral moderate pleural effusions, honeycombing with partial atelectasis of the underlying lung, and cardiomegaly. Computed Tomography Pulmonary Angiography (CTPA) showed findings likely consistent with probable mild pulmonary hypertension. An antinuclear antibody panel was performed and was positive for anti-U1-RNP, SS-A, SS-B, and Jo-1 antibodies. She was started on Lasix 40 mg, Aldactone 25 mg, lisinopril 10 mg, and heparin. Treatment with 20 mg of prednisolone was started and gradually tapered. Her anasarca and dyspnea improved, and she was discharged with follow-up arranged with cardiology and rheumatology. Timely identification of MCTD-associated pulmonary hypertension and its cardiovascular complications is essential and allows early commencement of treatment, which further prevents the patients from developing heart failure.
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BibTeX
@article{alla2026diagnostic,
title = {{Diagnostic Pitfall: Mixed Connective Tissue Disease-Associated Pulmonary Hypertension Presenting With Postpartum Right-Sided Heart Failure in a 21-Year-Old-Case Report.}},
author = {Sai Santhosha Mrudula Alla and Moksh Sanjay Khetan and Deekshitha Alla and Sri Lakshmi Ganguru and Srinidhi Appala and Krithi Sree and Riva Jayesh Shah and Aryan Seth and Nishant Anand Rawtani and Mohammed Raihanuddin Aamir and Nyassiri Warai Emmanuel},
journal = {Clinical case reports},
year = {2026},
month = nov,
doi = {10.1002/ccr3.73891},
url = {https://europepmc.org/article/MED/42999820},
note = {CC BY},
}
RIS
TY - JOUR
TI - Diagnostic Pitfall: Mixed Connective Tissue Disease-Associated Pulmonary Hypertension Presenting With Postpartum Right-Sided Heart Failure in a 21-Year-Old-Case Report.
AU - Sai Santhosha Mrudula Alla
AU - Moksh Sanjay Khetan
AU - Deekshitha Alla
AU - Sri Lakshmi Ganguru
AU - Srinidhi Appala
AU - Krithi Sree
AU - Riva Jayesh Shah
AU - Aryan Seth
AU - Nishant Anand Rawtani
AU - Mohammed Raihanuddin Aamir
AU - Nyassiri Warai Emmanuel
PY - 2026
DA - 2026/11/07
JO - Clinical case reports
DO - 10.1002/ccr3.73891
UR - https://europepmc.org/article/MED/42999820
AB - Mixed connective tissue disease is a multisystem autoimmune condition characterized by overlapping clinical and serological features related to anti-U1-RNP antibodies of at least two connective tissue diseases. It may present with myocarditis, pericarditis, pulmonary arterial hypertension, and, less commonly, heart failure. We present a 21-year-old female with bilateral foot swelling, cough, and shortness of breath 3 months after a vaginal delivery. On examination, she was hypotensive (90/70 mmHg), with an elevated jugular venous pressure, a pulmonary systolic murmur with loud P2, and a tricuspid regurgitation murmur. Bronchial breath sounds and fine crepitations were present in both lower lung fields. She was evaluated initially for suspected postpartum cardiomyopathy. Transthoracic echocardiography was suggestive of a probable moderate to severe PAH. High-resolution computed tomography showed bilateral moderate pleural effusions, honeycombing with partial atelectasis of the underlying lung, and cardiomegaly. Computed Tomography Pulmonary Angiography (CTPA) showed findings likely consistent with probable mild pulmonary hypertension. An antinuclear antibody panel was performed and was positive for anti-U1-RNP, SS-A, SS-B, and Jo-1 antibodies. She was started on Lasix 40 mg, Aldactone 25 mg, lisinopril 10 mg, and heparin. Treatment with 20 mg of prednisolone was started and gradually tapered. Her anasarca and dyspnea improved, and she was discharged with follow-up arranged with cardiology and rheumatology. Timely identification of MCTD-associated pulmonary hypertension and its cardiovascular complications is essential and allows early commencement of treatment, which further prevents the patients from developing heart failure.
SN - 2050-0904
ER -