✅ Partial muscular inter-ventricular septal defect (VSD) or Pacman heart is a rare congenital or occasionally acquired anomaly. Concurrent Pacman heart and Shone's complex are extremely rare and have never been reported until now.
Shone's complex is a rare congenital anomaly characterized by sequential obstructive lesions at multiple levels of the left heart structures and aorta (1). Partial ventricular septal defect (VSD) also known as Pacman heart is a rare cardiac abnormality that may be congenital or occasionally acquired (2). This abnormality is often clinically silent and is diagnosed incidentally when echocardiography is carried out to evaluate simultaneous structural heart diseases. The combination of Pacman anomaly and Shone’s complex is extremely rare. We described a case of congenital Pacman heart associated with flail mitral valve (FMV) and a partial form of Shone's complex comprising subvalvular aortic stenosis (AS), bicuspid aortic valve (AV), and coarctation of the aorta. Two-dimensional (2D) transthoracic echocardiography (TTE) is the imaging modality of choice for the diagnosis of Pacman. The incremental data are provided by three-dimensional (3D) and transesophageal echocardiography (TEE) regarding simultaneous structural heart diseases (3).
Discussion
Partial VSD is included in the differential diagnosis of complete VSD, left ventricular non-compaction, and focal hypertrophic cardiomyopathy. Accordingly, accurate diagnosis using cardiac imaging modalities is rather important since it prevents unnecessary surgeries and treatments. Because partial VSD is rare, reporting it, describing its association with Shone’s complex, and sharing echocardiographic images will assist cardiologists in making a proper diagnosis and treatment plan.
The previous case reports described the Pacman anomaly associated with mitral valve prolapse, bicuspid aortic valve and Parachute mitral valve. The combination of Pacman heart and Shone's complex is extremely rare and has not been reported until now.
The Pacman anomaly appears as a partial defect of the muscular portion of the interventricular septum, which changes size during systole and diastole (4). It is often clinically silent and is diagnosed incidentally using TTE. The complications of the Pacman deformity, such as myocardial rupture and conduction disturbance, are very rare (5), therefore, cardiac surgery is not recommended for this kind of partial defect. Cardiac surgery which was carried out for this patient was due to the severe MR and moderate AR but, the surgeon did not repair the partial VSD.
Conclusion
Due to the absence of a left-to-right shunt of this partial defect, cardiac surgery is not recommended.
Acknowledgements
None.
Conflicts of Interest
The authors declare that there is no conflict of interest.
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