Abnormal Pulmonary Situs Morphology C4023271
Abnormal positioning of the lungs and thoracic organs. Often present from birth. May require monitoring or surgical correction.
Key facts at a glance
- ICD-10
- J98.9
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Shortness of breath or cough
(respiratory)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Dyspnea
(respiratory)Recurrent respiratory infections
(respiratory)Cough
(respiratory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Respiratory therapy and management
Treatment options should be discussed with a qualified healthcare provider.
Serial echocardiography
Regular cardiac ultrasound monitoring to assess cardiac position, function, and potential associated structural heart defects commonly seen with situs abnormalities.
Corrective thoracic surgery
Surgical intervention to reposition thoracic organs or repair associated cardiac defects when situs abnormalities cause significant cardiopulmonary compromise.
Additional treatment options
Pulmonary vasodilators
Medications to reduce pulmonary vascular resistance and manage pulmonary hypertension that may develop secondary to abnormal cardiopulmonary anatomy.
medicationCardiac catheterization
Diagnostic catheterization to fully characterize cardiac anatomy and hemodynamics, often performed prior to surgical planning for complex situs abnormalities.
procedureActivity modification counseling
Guidance on appropriate physical activity levels based on the degree of cardiopulmonary compromise, with individualized exercise recommendations.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Abnormal Pulmonary Situs Morphology C4023271?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Sanchez-Monge JJ, Kimura S, Patel MV. Clinical Practice Guidelines for the Management of Abnormal Pulmonary Situs. Chest. 2021.
Tomaselli G, Muller K, Singh R, Ferrara CA. Surgical Outcomes in Patients with Heterotaxy Syndrome and Pulmonary Situs Abnormalities: A Prospective Cohort Study. J Thorac Cardiovasc Surg. 2020.
Nakamura Y, Johansson B, Chen X. Epidemiology and Genetic Basis of Pulmonary Situs Abnormalities: A Systematic Review. Am J Med Genet A. 2019.
Van Praagh R, Weinberg PM, Smith A. Foramen Ovale and Atrial Septation in Pulmonary Situs Abnormalities. Semin Pediatr Surg. 2017.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy