Double-inlet Left Ventricle with Situs Inversus: A Rare Congenital Cardiac Disease

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Double-inlet left ventricle with situs inversus totalis, mitral atresia, and severe pulmonary stenosis (n=1). Intervention: Conservative medical management with diuretics and antibiotics. Primary outcome: Symptom resolution and clinical stabilization prior to surgical referral.

Q: What does this research mean for the field?

Severe pulmonary stenosis can shield the pulmonary vascular bed from systemic hypertension and permit survival into adulthood in patients with double-inlet left ventricle and situs inversus. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Swarnadip Hazra; Rahul K Adhikary; Nilasish Dutta; Indranil Sen

doi:10.5005/ijcmd-11040-0025

October 28, 2026Open Access

Double-inlet Left Ventricle with Situs Inversus: A Rare Congenital Cardiac Disease

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Key result

Conservative management temporarily resolves NYHA IV symptoms in adult complex univentricular congenital heart disease.

n=1

Key Points

To describe the clinical presentation and echocardiographic characteristics of double-inlet left ventricle coexisting with situs inversus totalis and severe pulmonary stenosis in an adult female.
Clinical and diagnostic evaluation of a 30-year-old female presenting with NYHA class IV dyspnea, cyanosis, and fever.
Diagnostic confirmation performed using digital chest radiography, thoracic high-resolution computed tomography, and transthoracic echocardiography.
Transthoracic echocardiography demonstrated situs inversus, dextrocardia, L-loop, right-sided mitral atresia, an atrial septal defect with a right-to-left shunt, and a double-inlet left ventricle.
Both great arteries arose from the morphological left ventricle, with severe pulmonary stenosis demonstrating a pressure gradient of 66 mm Hg.
Conservative management using diuretics and antibiotics provided temporary symptom resolution prior to surgical referral.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

A 30-year-old female in India presenting with NYHA class IV dyspnea, cyanosis, and clubbing, diagnosed with double-inlet left ventricle, mitral atresia, severe pulmonary stenosis (66 mm Hg gradient), and situs inversus totalis.

I

Intervention

Conservative medical management with diuretics and antibiotics, followed by referral for heart transplantation or corrective surgical intervention.

O

Outcome

Temporary resolution of symptomspatient reported

This case report highlights a rare coexistence of double-inlet left ventricle, mitral atresia, and situs inversus totalis in an adult who survived to age 30 due to the protective effect of severe pulmonary stenosis shielding the pulmonary vasculature.

Implication

Temporary stabilization possible with diuretics in decompensated adult congenital cases; leaves open durability and generalizability in unoperated univentricular hearts.

Abstract

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Discussion

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Cite This Study

Hazra et al. (2026) conducted a case report in Double-inlet left ventricle with situs inversus totalis, mitral atresia, and severe pulmonary stenosis (n=1). Conservative medical management with diuretics and antibiotics was evaluated on Symptom resolution and clinical stabilization prior to surgical referral. Conservative management with diuretics and antibiotics temporarily resolved NYHA class IV symptoms in a 30-year-old female with double-inlet left ventricle, situs inversus, and pulmonary stenosis.

synapsesocial.com/papers/6ae682bde432e7a5c4ef3731 https://doi.org/10.5005/ijcmd-11040-0025

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Double-inlet Left Ventricle with Situs Inversus: A Rare Congenital Cardiac Disease

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

Double-inlet Left Ventricle with Situs Inversus: A Rare Congenital Cardiac Disease

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

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