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Splanchnectopia Accompanied With Atrial Septal Defect: A Case Report.

Ning Bao¹, Yu Zhang²

¹Medical undergraduate, Jilin University, Jilin province, China.

Cardiology Research

|March 30, 2017

Summary

Splanchnetopia with atrial septal defect is a rare congenital condition. Early diagnosis is crucial as symptoms may present atypically, potentially leading to misdiagnosis and delayed treatment.

Keywords:

Atrial septal defect Misdiagnosis Pneumonia Splanchnectopia

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Area of Science:

Cardiology
Medical Genetics
Thoracic Medicine

Background:

Splanchnetopia, a rare congenital malformation involving organ displacement, is infrequently associated with atrial septal defects.
Patients with this combination are increasingly recognized as susceptible to pulmonary complications.
This case highlights the diagnostic challenges posed by atypical presentations.

Observation:

A 62-year-old male presented with cough, palpitations, and dyspnea, exhibiting cyanosis, jugular venous distension, abnormal lung sounds, irregular heart rhythm, hepatomegaly, and lower extremity edema.
Diagnostic workup revealed leukocytosis with neutrophilia, atrial flutter on ECG, a significant atrial septal defect, dextrocardia, pulmonary artery abnormalities, and pneumonia on chest X-ray.
Abdominal ultrasound confirmed situs inversus (organ flip).

Findings:

The patient was diagnosed with congenital heart disease, splanchnetopia, atrial septal defect, Eisenmenger's syndrome, atrial flutter, New York Heart Association (NYHA) class III heart failure, and pneumonia.
The physical signs and symptoms were incongruent with typical presentations due to the organ displacement.

Implications:

This case underscores the importance of considering splanchnetopia in patients with congenital heart disease, especially when clinical signs are atypical.
Systematic and thorough physical examinations are essential to avoid misdiagnosis and ensure timely, appropriate treatment for such complex conditions.
Recognizing the interplay between congenital malformations and secondary complications like pulmonary disease is critical for patient management.