Congenital Anomalies

Partial or complete absence of the pericardium ( Fig. 10.1 ) is a rare condition that is often recognized as an incidental finding on a chest x-ray or evaluation of an abnormal ECG axis. Infrequently, partial absence of the pericardium may cause herniation of a cardiac chamber resulting in symptoms of atypical chest pain, dyspnea, or syncope. Ventricular dysrhythmias and sudden cardiac death rarely occur due to strangulation of the coronary vessels. Associated congenital cardiac defects include atrial and ventricular septal defects, bicuspid aortic valve, Tetralogy of Fallot, and patent ductus arteriosus. Pectus excavatum is also frequently found. Most frequently, congenital absence of the pericardium is partial, usually involving the left side of the heart, resulting in posterior and right axis deviation on the ECG. Complete absence is often associated with RV dilatation and secondary tricuspid regurgitation. Reproduced from a CMR obtained from a patient with complete congenital absence of the pericardium shows the absence of a parietal pericardial layer anterior to the RV ( arrow ) and displacement of the cardiac chambers toward the left hemithorax.

Steady-state free precession cardiac magnetic resonance image obtained from a patient with complete congenital absence of the pericardium. Notice the absence of a parietal pericardial layer anterior to the right ventricular ( arrow ) and the displacement of the cardiac chambers toward the left hemithorax.

Pericardial cysts are most often congenital in origin but may also develop after infection, chest surgery, or trauma. They represent the most common primary pericardial mass. Congenital pericardial cysts appear as smooth saccular structures generally with one lobe, ranging from 1 to 5 cm in diameter and filled with a transudate ( Fig. 10.2 , Videos 10.1A and 10.1B ). They often present as incidental findings or during the evaluation of atypical chest pain or dyspnea. They are usually located in the right costophrenic angle, the left costophrenic angle, or the anterior mediastinum. A pericardial diverticulum may mimic the imaging features of a pericardial cyst; however, communication with the pericardial space is the distinguishing feature of a pericardial diverticulum.

Typical appearance of a pericardial cyst. Pericardial cyst. (A and C) Posteroanterior and lateral chest radiography revealed a well-circumscribed homogenous opacity at the right anterior costophrenic angle ( arrows ). (B) Modified apical four-chamber view by echocardiography demonstrating a well-defined hypoechoic mass ( asterisk ) adjacent to the right atrium. (D) Axial view of contrast enhanced computed tomography with soft tissue windows demonstrating a homogenous, low attenuation well-circumscribed mass ( asterisk ) in the right costophrenic angle adjacent to the right atrium.

Reproduced from Tower-Rader A, Kwon D. Pericardial masses, cysts and diverticula: a comprehensive review using multimodality imaging. Prog Cardiovasc Dis . 2017;59(4):389–397.

The noninvasive imaging characteristics found in common congenital pericardial anomalies are listed in Table 10.1 .

Pericardial Effusion

A small amount of pericardial fluid may be visualized in asymptomatic normal subjects. Pathological fluid accumulation may occur in inflammation, trauma, or malignancy. Large pericardial effusions may be suspected on chest x-ray films. However, most pericardial effusions are detected by echocardiography in the clinical setting. Images and videos obtained from three patients evaluated for dyspnea demonstrate the distinct echocardiographic characteristics of a pericardial effusion ( Fig. 10.2A , Video 10.2A ), a pleural effusion ( Fig. 10.2B , Video 10.2B ), and ascites ( Fig. 10.2C , Video 10.2C ). Posterior pericardial effusions appear anterior to the descending aorta, whereas pleural effusions are located posteriorly. Ascites are most often seen from the subcostal acoustic window below the diaphragm. Both CT and MRI can detect even small amounts of pericardial fluid and compared to echocardiography, may more accurately quantify the amount of fluid. Large effusions may appear as a zone void of radiotracer around the cardiac silhouette in nuclear scintigraphy studies ( Video 10.3 ). The association between the size of pericardial effusion, symptoms, and hemodynamic effect, depends on the etiology and the rate of fluid accumulation. In acute trauma, a small amount (25–50 mL) of blood may result in a significant increase in intrapericardial pressure. Conversely, transudative pericardial effusions associated to metabolic, endocrine disorders, and hepatic failure may increase in volume gradually distending the pericardial space without causing significant hemodynamic compromise. Exudates, having high protein and cell content, have high intensity on T1 and low intensity on T2-weighted images ( Fig. 10.4 ). Hemorrhagic effusions are characterized by high signal intensity on T1-weighted images and inhomogeneous low signal intensity on cine SSFP images ( Fig. 10.5 ). On CT studies, transudative effusions have low attenuation (0–10 HU), whereas exudative effusions and blood have higher attenuation values.

Cardiac magnetic resonance in pericarditis . Serial cardiac magnetic resonance imaging (MRI) in a 17-year-old patient who had initially presented for evaluation of persistent symptoms due to acute pericarditis. (A) Index cardiac MRI using late gadolinium enhancement technique demonstrating active inflammation involving most of the pericardium ( red arrow ). B: Cardiac MRI of the same patient after 3 months of therapy with colchicine and steroids demonstrating resolution of inflammation ( red arrow ).

Reproduced from Baskar S, Betancor J, Patel K, et al. Cardiac MRI in evaluation and management of pediatric pericarditis. Prog Pediatr Cardiol . 2018;50:39–45.

Pericardial hematoma after blunt chest trauma . (A and B) Short- and long-axis cardiovascular magnetic resonance views, T1: double inversion recovery (IR) with contrast sequence obtained at presentation; (C and D) short- and long-axis views, T1: double IR with contrast sequence showing near complete resolution at 18 months.

Reproduced from Martína M, Santamartab E, Corros C. Pericardial hematoma after blunt chest trauma. Port J Cardiol . 2012;31(9):623–624.

(A) Transesophageal echocardiography (TTE) images and video obtained from a patient with a large posterior pericardial effusion. Notice that the effusion ends anterior to the descending aorta (Ao). (B) TTE images and video were obtained from a patient with a posterior pleural effusion. Notice that the effusion ends posterior to the descending Ao. A smaller pericardial effusion is also seen ( arrow ). (C) TTE images and video obtained from a patient with ascites (A) from a subcostal window. Notice the presence of the falciform ligament ( arrow ).

The noninvasive imaging characteristics found in transudative, exudative, and malignant pericardial effusions are listed in Table 10.2 .

Cardiac Tamponade

When a pericardial effusion results in a significant increase in pericardial pressure, venous return to the atrial chambers is compromised, resulting in increased central venous pressure, and decreased cardiac output. The classic features of cardiac tamponade in imaging studies reflect the hemodynamic alterations consisting of elevated central venous, reduced stroke volume, and ventricular interdependence. However, localized cardiac tamponade affecting a single cardiac chamber may present with partial or atypical features and be more difficult to diagnose. Establishing the diagnosis and guiding therapy in cardiac tamponade relies almost exclusively on Echocardiography. Two-dimensional imaging is used to identify the presence of chamber collapse ( Fig. 10.6 , Video 10.4 ). With increasing levels of intrapericardial pressure, first the RA, followed by the RV, LA, and in extreme situations the LV-free walls, will invaginate during diastole. The IVC will increase in diameter and remain distended during force inspiration, indicating elevated RA pressure. Pulsed Doppler recordings will demonstrate exaggerated variability during the respiratory cycle. It is important to consider that these diagnostic features may be diminished in hypovolemia or exaggerated in respiratory failure or during mechanical ventilation.

Transesophageal echocardiography images and video obtained from a patient in cardiac tamponade. There is a moderate-sized circumferential pericardial effusion and evidence of right atrium wall inversion ( arrow ).

The noninvasive imaging characteristics found in cardiac tamponade are listed in Table 10.3 .

Table 10.3

Imaging Findings in Cardiac Tamponade, Constrictive and Effusive-Constrictive Pericardial Disease

	Cardiac Tamponade	Pericardial Constriction	Effusive-Constrictive
Chest x-rays	• Enlarged cardiac silhouette, “water bottle” appearance if large effusion present	• Normal heart size or enlarged atria, pericardial calcification seen in 25%–50% • Pleural effusions may be present	• Enlarged cardiac silhouette if large effusion present • Pleural effusions may be present
Echocardiography	• Echo lucent space between visceral and parietal layers • Size of effusion varies depending on the rate of development • RA, RV, and/or LA wall inversion • Dilated, noncollapsing IVC • Exaggerated inspiratory increase (>40%) in RV filling Doppler E and/or decrease (>25%) in LV filling Doppler E	• Variable echo-density • Fibrin strands • Increased pericardial “brightness” • Variable pericardial thickness • Interventricular septal bounce • Dilated, noncollapsing IVC • Exaggerated inspiratory increase in RV filling Doppler E and/or decrease in LV filling Doppler E • Annulus reversus (septal>lateral TDI e’)	• Variable pericardial thickness and fluid density • Irregular appearance • Possible myocardial, intracardiac, and/or extracardiac involvement • Exaggerated respiratory variability and IVC plethora that persists after pericardiocentesis
Computed tomography	(Incidental finding) • < 2 mm pericardial thickness • 4 mm effusion diameter • Fluid attenuation 0–10 HU • Dilated IVC, contrast reflux	• Variable pericardial thickness • Contrast enhancement on delayed imaging • Fluid attenuation > 10 HU • Dilated IVC, contrast reflux	• Variable and irregular pericardial thickness • Fluid attenuation >10 HU • Early phase contrast enhancement if high vascularity
Magnetic resonance imaging	Not indicated	• Variable pericardial thickness • 4 mm effusion diameter • Dilated IVC • High signal intensity on T1W • Low signal intensity on T2W • LGE presence suggests active inflammation and a possible response to medical therapy	• Variable signal intensity on T1W and T2W depending on tumor vascularity • Irregular pericardial thickness • LGE may be present
Nuclear scintigraphy	Not indicated	• Present pericardial 18-FDG uptake suggests active inflammation and a possible response to medical therapy	• 18-FDG uptake may be present

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