Multimodality Imaging Approach Evaluation of the Congenital Pericardial Defect: a Contemporary Review.

PURPOSE OF REVIEW: This review presents the etiology, clinical manifestations, diagnostic approach, and treatment of congenital pericardial defects. It also highlights the critical role of echocardiography, cardiac computed tomography (CCT), and cardiac magnetic resonance (CMR) in the diagnosis and management approach. RECENT FINDINGS: Congenital pericardial defects are rare. Although most cases are found incidentally, some cases could potentially be associated with serious outcomes including sudden cardiac death. The diagnosis is often challenging due to non-specific clinical manifestations and electrocardiogram findings. Echocardiography is the first-line imaging investigation for the evaluation of this condition. Advanced cardiac imaging modalities, including CCT and CMR, play important adjuvant roles in establishing the diagnosis and assists with prognostication.

Congenital partial pericardial defect discovered incidentally during surgery for lung cancer: a case report and literature review.

BACKGROUND: Congenital pericardial defects are rare congenital anomalies, often asymptomatic and incidentally detected during thoracic surgery. CASE PRESENTATION: A 74-year-old man with primary lung cancer (cT1cN0M0, Stage IA3) underwent thoracoscopic radical lobectomy. At the time of thoracotomy, a pericardial defect was found on the ventral side of the hilar region, and the left atrial appendage was exposed. Due to concern that contact between the bronchial stump and the left atrial appendage may lead to postoperative bleeding and heart hernia, the pericardial defect was closed with an expanded polytetrafluoroethylene GoreTex® membrane. Preoperative computed tomography was reanalyzed with a 1 mm slice, congenital pericardial defect was detected as the pericardium had penetrated under the left atrial appendage. CONCLUSIONS: In congenital partial pericardial defect, contact between the left atrial appendage and bronchial stump, due to movement of the lung or heart, increases the risk of bleeding after lung resection. Therefore, closure of the defect should be considered. Although it is difficult to diagnose congenital partial pericardial defect preoperatively, computed tomography taken with a slice thickness of 1 mm is useful for diagnosis.

[Partial pericardium defect with a cardiac heart diverticulum and extensive intrauterine hypoxic myocardial lesions]. / Partieller Perikarddefekt mit herniertem Herzdivertikel und ausgedehnten intrauterinen hypoxischen Myokardschädigungen.

The autopsy of a fetus at 23 weeks gestational age revealed a partial pericardial defect with subsequent herniation of parts of the left ventricle. The myocard was impinged by the fibrous rim of the residual pericardium. Microscopic examination showed signs of recurrent myocardial infarctions with necrosis, calcification, fibrosis and prominent deposition of iron.Partial pericardial defects result from incomplete fusion of the pleuropericardial membrane and may lead to myocardial infarction via compression of the coronary arteries.

Right Ventricle Mimics Right Atrium at First Glance: A Rare Case of Congenital Right Sided Partial Pericardial Defect.

Among heart irregularities, congenital pericardial defect is an unusual anomaly, and is typically left dominant. However, cases of right pericardial defect with heart herniation are extremely rare. This is a case of congenital right pericardial defect with herniation of the right ventricle free wall and right ventricular outflow tract. The patient is asymptomatic and refused further intervention but even indolent discomfort underscores the risks of iatrogenic injuries to the heart and sudden death caused by mechanical pathogenesis due to changes in anatomical positions of the cardiac structures.

Congenital partial defect of the left pericardium presenting as chest pain.

Congenital pericardial defect is a rare cardiac defect with variable clinical presentations. It is usually an unexpected finding during cardiac surgery or autopsy. The clinical detection of congenital absence of pericardium is important because of its life-threatening complications such as fatal myocardial strangulation, myocardial ischaemia and sudden death. We present a patient with the incidental finding of left-sided partial defect of the pericardium during evaluation of chest pain.

A Case of Incidentally Discovered Congenital Complete Pericardial Defect during Lobectomy for Lung Cancer: A Case Report and Literature Review.

An 82-year-old male patient underwent a left upper lobectomy with anterolateral thoracotomy for lung cancer. Although a complete left-pericardial defect was observed during surgery, the pericardial repair was not performed because the left lower lobe remained and the heart was considered stable. Postoperative pathological examination revealed primary synchronous double-lung squamous-cell carcinoma (pathological stage pT2a(2)N0M0 stage IB). He was discharged without complications on postoperative day 8. Leftward displacement of the heart and left diaphragmatic elevation, suspected of phrenic-nerve paralysis, were found in the chest X-ray after discharge. However, the patient's overall condition remained unaffected at the 5-month postoperative follow-up. To assess the need for pericardial repair, we compared cases of complete pericardial defects observed during lobectomy or pneumonectomy reported in the literature. Only one of 12 cases occurred postoperative death despite pericardial repair, and that case combined pectus excavatum and pericardial defects. Our assessment indicated that pericardial repair might not be necessary, excluding complex cases.

Left lower lobectomy without pericardial reconstruction in a patient with a congenital pericardium defect.

Pericardial defects are rare congenital disorders. We report a case of a left lower lobectomy in a patient with lung cancer, a congenital complete left-sided pericardial defect and severe pleural adhesions. The pleural adhesions between the epicardium and lungs were carefully dissected. A left lower lobectomy with mediastinal nodal dissection was performed under complete video-assisted thoracoscopic surgery without pericardial reconstruction. The patient remained asymptomatic for 20 months postoperatively. Careful dissection of severe adhesions is necessary in patients with severe cardiac pulsations.

Left ventricular herniation and incarceration with infarction secondary to a pericardial defect in a dog.

A 2-year-old spayed female Labrador Retriever with clinical signs of heart failure presented to necropsy with a pericardial defect with most of the left ventricle non-reducibly herniated into the pleural space. The herniated cardiac tissue was constricted by a ring of pericardium that allowed subsequent infarction, evidenced by a marked depression on the epicardial surface. A congenital defect was considered more likely than a traumatic cause, since the margin of the pericardial defect was smooth and fibrous. Histologically, the herniated myocardium was acutely infarcted while the epicardium at the defect margin was markedly compressed, including the coronary vessels. This appears to be the first report of ventricular cardiac herniation with incarceration and infarction (strangulation) in a dog. Similar cardiac strangulations may rarely occur in humans with congenital pericardial defects or acquired pericardial defects secondary to blunt trauma or thoracic surgery.

Left upper lobectomy in a patient with congenital pericardial defect.

Congenital pericardial defect is an uncommon anomaly due to the congenital absence of the pericardium. In this case, it was associated with diaphragmatic hernia and incidentally discovered during thoracoscopic left upper lobectomy for lung cancer. The thoracoscopic dissection of the hilar structures was complicated with arrhythmia and hypotension. Thus, we converted thoracoscopy to thoracotomy, and the lobectomy was successfully performed. The pericardial defect and diaphragmatic hernia were not repaired, but the vessels and bronchial stump were covered to avoid heart injury. Postoperative course was unremarkable and at six-month follow-up patient did not have any problems.

Right pneumothorax with congenital pericardial defect showed right atrium mimicking bulla in surgery.

BACKGROUND: Congenital pericardial defect (CPD) is found incidentally in cases of pneumothorax. CPD is seen in left side rather than right side and it is not generally known among thoracic surgeons how the inside of the pericardial space can be seen from the thoracic cavity in cases of pericardial defect. CASE PRESENTATION: A 52-year-old man with dyspnea was referred to our hospital because of the diagnosis of right pneumothorax. Chest radiography showed a right lung collapse and a pneumopericardium on the left side. Despite insertion of a chest tube, air leakage prolonged, bullectomy at the apex of the right lung was performed under thoracoscopy. During surgery, thoracoscope showed that the right atrium seemed as if it had been a non-pedunculated bulla or cardiac cyst. Heart beating, continuity with the heart, and the absence of respiratory motion could distinguish the right atrium from a bulla, and pericardial defect was confirmed. Preoperatively, the patient had no cardiac symptoms related to the CPD, and therefore, it was determined that a procedure to close the CPD was not necessary. Any complication and recurrence did not occur 6 months after surgery. CONCLUSIONS: Right pneumothorax with CPD showed right atrium mimicking bulla in surgery. It is important to consider correction of CPD if there are cardiac symptoms at the onset of pneumothorax, and not to misinterpret the right atrium as a bulla.

An Incidental Finding of a Large Pericardial Cyst.

A pericardial cyst is a rare and benign cause of a mediastinal mass. They are frequently asymptomatic and are usually incidental findings on imaging. Symptoms may include persistent cough, atypical chest pain, dysphagia, and dyspnea. Diagnosis is usually established with the aid of imaging, including a chest x-ray, a computed tomography (CT) scan, and magnetic resonance imaging (MRI). Therapeutic options include surgical resection or aspiration for large and/or symptomatic cysts, whereas conservative management with routine follow-up is advised for small or asymptomatic cysts. We herein describe the case of a 48-year-old lady, who presented with clinical features suggestive of acute cholecystitis, with an incidental finding of a pericardial cyst, measuring approximately 10.1 cm x 8.7 cm x 10.7 cm. The patient underwent video-assisted thoracoscopic surgery (VATS) for excision of the pericardial cyst. She had an uneventful recovery and was discharged on the second post-operative day. At six months, there was no evidence of disease recurrence.

Iatrogenic pericardial defect.

Pericardial defects are uncommon, usually congenital, and frequently involve a complete absence of the pericardium. Partial pericardial defects are more likely to result in complications. Iatrogenic pericardial defects are usually partial defects and may present with cardiac strangulation. We present the case of an iatrogenic pericardial defect in an asymptomatic 20-year-old female.

Incidental finding of congenital pericardial defect during vats bullectomy. Tips and tricks to avoid blunder.

The absence of a small portion of the pericardium is termed pericardial defect. This rare entity can be either acquired or congenital. The prevalence of congenital pericardial defect is exceedingly rare, which is approximately 0.002-0.004% of surgical and pathologic series. The most common type is the absence of the entire left side of pericardium, seen in 67% of all patients with a congenital pericardial defect. Other varieties are incredibly uncommon. Congenital pericardial defect has a male preponderance with a male to female ratio of 3:1, and familial occurrence is uncommon. We report a case of left partial congenital pericardial defect detected incidentally in a 22-year-old man who presented with recurrent left spontaneous pneumothorax. He underwent video-assisted thoracoscopic bullectomy and intraoperatively, we discovered a left partial pericardial defect which exposed the left atrial appendage. Although generally asymptomatic, patients may present with non-specific cardiac symptoms such as atypical chest pain. Partial pericardial defects have an increased risk of herniation of the whole left atrium, the left atrial appendage or the ventricles. If this occurs, cardiac strangulation may occur, leading to necrosis and sudden death. Cardiac MRI is a sensitive tool and will demonstrate the absence of preaortic pericardial recess. In conclusion, no surgical intervention is required in cases of congenital pericardial defect, unless the patient is symptomatic due to complications. If detected incidentally during cardiac or thoracic surgery, the best may be to leave it alone.

Mature mediastinal bronchogenic cyst with left pericardial defect: A case report.

BACKGROUND: Mediastinal bronchogenic cysts and pericardial defects are both rare. It is extremely rare that both occur simultaneously. To the best of our knowledge, this is the first case of a coexistent bronchogenic cyst and pericardial defect reported in China. We performed a literature review and found a relationship between bronchogenic cysts and pericardial defects, which further revealed the correlation between the bronchus and pericardium during embryonic development. CASE SUMMARY: A 14-year-old boy attended a local hospital for ankylosing spondylitis. Chest radiography showed an enhanced circular-density shadow near the left mediastinum. The patient had no chest symptoms and the physical examination was normal. Because of the mediastinal occupation, the patient visited our department of chest surgery for further treatment. During surgery, a left pericardial defect was observed. The bronchogenic cyst was removed by thoracoscopic surgery, but the pericardial defect remained untreated, and a satisfactory outcome was achieved after the operation. The patient was diagnosed with a mediastinal tumor. The pathological diagnosis of the tumor was a bronchogenic cyst. CONCLUSION: This case further reveals the correlation between the bronchus and pericardium during embryonic development.

Congenital Partial Absence of Pericardium in an Elderly Patient.

Patients with a complete absence of pericardium require no intervention as they are mostly asymptomatic. Due to the risk of herniation, patients with partial absence of pericardium tend to present with symptoms and may benefit from treatment. We report a case of an elderly patient who presented for severe colitis and was incidentally found to have a partial absence of the pericardium on the right side of the heart.

Cardiac Strangulation Due to Partial Pericardial Defect Presenting as Acute Myocardial Infarction.

A 79-year-old man with chest pain and dyspnea underwent emergency percutaneous coronary intervention for acute myocardial infarction. However, he died 17 days later due to refractory heart failure. An autopsy revealed cardiac strangulation caused by herniation of the apical heart through a pericardial defect due to partial absence of the pericardium. (Level of Difficulty: Advanced.).

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

INTRODUCTION: Congenital pericardial defects are rare but can cause fatal complications. Most cases are asymptomatic and incidentally detected during a thoracic surgery or autopsy. We report a case of a partial pericardial defect confirmed based on spontaneous pneumothorax. PRESENTATION OF CASE: A 16-year-old boy with left spontaneous pneumothorax showed pneumopericardium on chest X-ray. Chest computed tomography revealed a partial pericardial defect. Video-assisted thoracoscopic surgery was performed, and a small pericardial defect was confirmed at the level of the upper pulmonary hilum. We did not reconstruct the defect because of the improbability of cardiac herniation. DISCUSSION: Pneumopericardium combined with pneumothorax suggests the existence of a pericardial foramen. Partial pericardial defects could cause cardiac herniation or strangulation, and pneumothorax may worsen the protruding of the heart. Video-assisted thoracic surgery is an effective method to prevent the recurrence of pneumothorax and determine whether reconstruction of the defect is required. CONCLUSION: Video-assisted thoracic surgery should be performed in the case of a pericardial defect combined with pneumothorax.

Relief of cardiac tamponade by a congenital partial left-sided pericardial defect in a patient with ruptured acute type A aortic dissection: a case report.

BACKGROUND: Acute type A aortic dissections have an extremely poor prognosis, and cardiac tamponade is a major cause of death in these patients. Here, we describe a case where congenital partial pericardial defect relieved cardiac tamponade caused by ruptured type A aortic dissection. CASE PRESENTATION: A 79-year-old woman was hospitalized after experiencing chest pains and respiratory distress. She developed out-of-hospital cardiopulmonary arrest and was resuscitated with no sequelae 5 days before admission. Computed tomography confirmed pericardial and left pleural effusions, and type A aortic dissection was diagnosed. We began emergency ascending aortic replacement surgery under general anesthesia with propofol and remifentanil and incidentally discovered a congenital partial left-sided pericardial defect that allowed drainage of the hemopericardium and relieved cardiac tamponade. The surgery was successfully performed, and the patient recovered without complications. CONCLUSIONS: We experienced an extremely rare case where a congenital partial pericardial defect relieved cardiac tamponade associated with aortic dissection and contributed to the patient's survival.

Sometimes Surgery is the Only Way to Make a Diagnosis and Treat the Patient: A Case of Congenital Partial Absence of Pericardium.

INTRODUCTION: A 47-year-old Caucasian woman with a past medical history of multiple ablative procedures for supraventricular arrhythmias and pacemaker implantation presented with increasing shortness of breath. The initial working diagnosis of the team treating her was ablation-induced pulmonary stenosis, especially after the recording of increased flow velocities through the right lower pulmonary vein. CASE PRESENTATION: The patient was alert and oriented, but obviously dyspnoeic. The vital signs were normal. The physical examination revealed a soft cardiac systolic murmur and the lungs were clear on auscultation. The electrocardiogram showed a pacemaker rhythm. The echocardiogram showed borderline normal global systolic function of the left ventricle and severe mitral regurgitation. The transoesophageal echocardiogram confirmed the above findings and revealed increased velocities through the right lower pulmonary vein. The working diagnosis of ablation-induced pulmonary stenosis was reinforced by the cardiac CT angiography. The patient was subsequently referred for surgical intervention. The intra-operative findings were both unexpected and impressive: congenital partial absence of the pericardium was responsible for herniation of the right chambers into the pleural space. Mitral regurgitation was attributed to failure of coaptation due to the very short surface of the leaflets. Extensive external fibrosis around the pulmonary veins caused the pulmonary vein stenosis. CONCLUSION: The final diagnosis of a partial pericardial defect causing torsion and distortion of the heart chambers was made only at surgery. The consistent finding of pulmonary vein stenosis in the non-invasive modalities and the past medical history of ablations initially misleadingly led us to the assumption that they were related. LEARNING POINTS: The initial working diagnosis of ablation-induced pulmonary vein stenosis based on the patient's shortness of breath, severe mitral regurgitation, right lower pulmonary vein stenosis and past medical history of multiple ablative procedures, was incorrect.The finding that the cardiac silhouette was borderline displaced to the right, the mildly hypoplastic right lung and the borderline impairment of the global systolic function of the left ventricle, especially after coronary artery disease was ruled out, should have been given greater consideration.Cardiac computer tomographic images taken 7 years ago were used to assess the severity of the right inferior pulmonary vein stenosis; new medical technologies will provide even better diagnostic techniques.

Congenital Absence of the Pericardium.

Congenital absence of the pericardium (CAP) is one of the rarest cardiac congenital anomalies. It can occur as a complete absence of the entire pericardium, absence of the right or left portion of the pericardium or a partial, foramen-like defect of the right or left pericardium. While the majority of cases are clinically silent, multiple reports associate CAP with symptomatic presentation. The most feared complication of CAP is sudden death due to cardiac strangulation across a partial defect of the left pericardium. Given its rare occurrence, most clinicians and imaging specialists will have little experience with this condition and may fail to recognize it on thoracic or cardiac studies. Thus, the purpose of this review is to highlight the common clinical and multimodality imaging features associated with this anomaly and suggest a management algorithm.