Differential Diagnosis for Rv Dilated with Dilated Main Pulmonary Artery with Mosaic B/L with Dextrocardia
- Single Most Likely Diagnosis
- Kartagener Syndrome: This is a subset of Primary Ciliary Dyskinesia (PCD) characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus (which includes dextrocardia). The mosaic pattern on imaging is suggestive of bronchiectasis and air trapping, common in PCD. RV dilation and a dilated main pulmonary artery can be seen due to pulmonary hypertension, a complication of chronic lung disease.
- Other Likely Diagnoses
- Cystic Fibrosis: While not typically associated with dextrocardia, cystic fibrosis can cause bronchiectasis and mosaic attenuation on imaging due to air trapping and bronchial wall thickening. Pulmonary hypertension can lead to RV dilation.
- Chronic Thromboembolic Pulmonary Hypertension (CTEPH): This condition can cause a dilated main pulmonary artery and RV dilation due to increased pulmonary vascular resistance. Mosaic perfusion can be seen on imaging due to uneven distribution of thromboemboli.
- Do Not Miss Diagnoses
- Pulmonary Embolism (PE): Although less likely to cause a mosaic pattern bilaterally, large or multiple PEs can lead to acute RV dilation and a dilated main pulmonary artery. Dextrocardia is not directly related but can be an incidental finding.
- Pulmonary Arterial Hypertension (PAH): Idiopathic or associated with other conditions, PAH can cause RV dilation and a dilated main pulmonary artery. Mosaic perfusion can be seen due to uneven vascular resistance.
- Rare Diagnoses
- Congenital Heart Disease with Pulmonary Hypertension: Certain congenital heart diseases (e.g., Eisenmenger syndrome) can lead to pulmonary hypertension, causing RV dilation and a dilated main pulmonary artery. Dextrocardia can be part of the congenital anomaly.
- Ivemark Syndrome: A rare condition characterized by congenital heart disease, hepatic fibrosis, and other anomalies, which can include dextrocardia. Pulmonary involvement might lead to the described imaging findings, although it's less common.