Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects

Abstract

ObjectiveCongenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%–0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.MethodsWe retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.ResultsEight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5–144.9 months) and all remained asymptomatic.ConclusionCPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.

Congenital partial pericardial defect confirmed based on spontaneous pneumothorax: A case report and literature review

“…It shows male predominance and mostly occurs on the left side. The partial absent type accounts for 24%–63% of all cases of confirmed pericardial defects [ 2 , 5 , 6 ]. Symptomatic patients typically complain of chest pain, dyspnea, and palpitation.…”

“…Symptomatic patients typically complain of chest pain, dyspnea, and palpitation. Approximately one-third of the patients have associated cardiac anomalies, such as an atrial septal defect, mitral stenosis, patent ductus arteriosus, and tetralogy of Fallot [ 5 , 6 ]. In the present case, echocardiography revealed no cardiac abnormalities.…”

“…Lateral displacement of the heart is a typical finding in patients with complete absence of the pericardium. Chest X-ray may show abnormal presence of the lung tissue between the aorta and the pulmonary artery or between the heart and the diaphragm [ 2 , 5 , 6 ]. CT or MRI should be considered for patients with a suspected or diagnosed pericardial defect to assess the size and location of the defect [ 7 , 8 ].…”

“…CT or MRI should be considered for patients with a suspected or diagnosed pericardial defect to assess the size and location of the defect [ 7 , 8 ]. Artificial pneumothorax is a histological diagnostic method, which is rarely used nowadays because of its invasiveness and morbidity [ 2 , 5 ]. Pneumopericardium following pneumothorax can prove the existence of a pericardial foramen, as shown in the present case.…”

“…Patients with complete or large absence of the pericardium, which allows the heart to move back smoothly into the cardiac space, usually need no interventional treatment and show favorable prognosis [ 6 ]. Small-to-moderate-sized defects should be considered for surgical treatment because of the possibility of ventricle or atrial appendage strangulation [ 2 , 5 ]. Bennett KR et al reviewed 44 reported cases, including five fatal cases, of partial pericardial defects [ 2 ].…”