A UNIQUE PRESENTATION AND TREATMENT OF A RARE CONDITION: CARDIAC POSTPNEUMONECTOMY SYNDROME

Objectives: Postpneumonectomy syndrome (PPS) is an extremely rare condition that occurs in a small percentage of patients who have undergone a pneumonectomy. The condition is characterized by bronchial malacia with intermittent obstruction caused by tethering of the remaining mainstem bronchus over the spine secondary to mediastinal shift. This condition affects mostly young women and presents as progressive dyspnea, shortness of breath and stridor. The objective of our case report is to highlight the unique anatomic changes seen in our patient 12 months after an intrapericardial pneumonectomy. Not only was the patient an adult male, but, the intrathoracic changes causing the patient’s symptoms were secondary to significant ipsilateral diaphragm atrophy and elevation with intrathoracic herniation of the liver which resulted in compression of the right atrium and ventricle with cardiopulmonary compromise.

The patient is a 56-year-old male who had stage IIIA atypical carcinoid of the right lung. He underwent a right intrapericardial pneumonectomy and resection of phrenic nerve secondary to tumor invasion. Six months after surgery, he started experiencing shortness of breath and worsening dyspnea on exertion. A 12-month follow-up CT scan of the chest demonstrated no evidence of recurrent cancer, but significant elevation of the diaphragm with near complete occupation of the right pleural cavity with the liver and abdominal contents. The patient’s symptoms were related to compression of the right atrium and ventricle and he was diagnosed with post pneumonectomy syndrome secondary to an atrophied diaphragm without bronchial abnormalities. The patient underwent a redo thoracotomy with reduction of intrathoracic contents, diaphragm plication and placement of intrathoracic saline tissue expander. One month after repair, the patient had complete resolution of his symptoms. Follow-up CT scan showed the tissue expander in an advantageous position without significant diaphragmatic elevation and without cardiac compression.

This case depicts a rare condition with an even rarer presentation. Our patient is a middle-aged male, whereas most patients are young females. The anatomic changes that caused the symptoms in our patient were secondary to an elevated atrophied diaphragm and intrathoracic viscera causing compression of the right side of the heart. Although our case is unique, the treatment chosen for our patient has been described as one of the most successful treatment options, which is, surgical correction with a prosthetic implant. This is the first reported case of PPS related to an atrophied diaphragm and intrathoracic viscera causing cardiac compression which was successfully treated with an intrathoracic prosthetic implant.

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