Synonyms: Pericardial hemorrhagic effusion.

Definition: Hemorrhage contained by the pericardium.

Prevalence: Nonimmune hydrops: 2.5-6.6:10,0000 deliveries. Hemopericardium: unknown.

Etiology: Unknown.

Pathogenesis: Hemorrhagic dia­thesis is hypothetic.

Associated anomalies: In the present case: hydrops, esophageal atresia with tracheoesophageal fistula, single umbilical artery, low set ears.

Differential diagnosis: Any hyperechoic intrathoracic mass, such as cardiac or pericardiac tumors, type III cystic adenomatoid malformations, thymic mass, teratoma...

Prognosis: Dependent upon associated anomalies and hydrops.

Recurence risk: Unknown.

Management: Dependent upon associated anomalies.

MESH Pericardial-Effusion,-Etiology ICD9 772.8 (hemopericardium in the newborn) CDC 746.850

Address correspondence to E. Fred Avni, MD, PhD Hospital Erasme, Department of Radiology, Route de Lennik 808, B1070 Brussels, Belgium. Ph: 32-2-555-3288; Fax: 32-2-555-4545

Introduction

Fetal pericardial effusion is a rare anomaly, usually easily diagnosed by sonograms. We describe an unusual form of pericardial effusion: a fetal hemopericardium.

Case report

A 27-year-old G₁P₀ woman presented at 26 weeks of gestation with premature labor and vaginal bleeding. Sonography revealed fetal hydrops with oligohydramnios (fig. 1).

Fig. 1: Ascites on an oblique scan of the abdomen.

The patient"s medical history was unremarkable except for her Rh system group, which was A^-. Indirect Coombs crossmatch and Kleihauer-Betke test were negative. The patient was transferred to our institution for cordocentesis.

On examination, a large pulsatile echogenic mass was found around the fetal heart without clear distinction between the mass and the myocardium (fig. 2).

Fig. 2: A hyperechoic shell surrounds the heart, from which it is poorly differentiated.

The color Doppler examination showed no flow in the hyperechoic mass (fig. 3).

Fig. 3: Color Doppler demonstrates normal flow in the heart. Note the hydrops (arrows).

Shortly after, the patient delivered a premature 832g female infant. The karyotype was normal. The autopsy revealed multiple anomalies such as hydrops, esophageal atresia with tracheo­esophageal fistula, single umbilical artery, low set ears, and blood under the tentorium cerebelli. The pericardial sac contained 50 ml of blood with clots. The fetal heart was otherwise normal. No cause could be found for this pericardial hemorrhagic effusion with non-immune hydrops.

Discussion

Prevalence

The incidence of non-immune hydrops is about 2.5-6.6:10,0000 deliveries, but hemorrhagic pericardial effusion has never been reported in a fetus and its prevalence is unknown¹.

Ultrasound appearance

In our case, the pericardial effusion presented as a large hyperechoic mass, poorly differentiated from the myocardium. The mass circumscribed the fetal heart and was pulsatile. The lungs were compressed.

Etiology

The etiology of hemopericardium in infants includes chest trauma either accidental or iatrogenic (i.e.: birth trauma, closed chest massage), generalized diathesis, pericardial teratomas, and rupture of cardiac aneurysms or diverticula². Although a birth trauma cannot be excluded, the presence of blood in the posterior fossa might suggest a bleeding diathesis in this case. No pericardial or cardiac lesion could be found at autopsy.

Associated anomalies

Hemopericardium, as any pericardial effusion, may induce cardiac tamponade with cardiac decompensation. This might explain the fetal hydrops in our case. Hemopericardium has been associated in children with pericardial teratoma, with cardiac aneurysm rupture, and with generalized bleeding diathesis². Furthermore, in our case, there were a single umbilical artery, esophageal atresia with tracheoesophageal fistula, and low set ears.

Differential diagnosis

The differential diagnosis includes other hyperechogenic cardiac and pericardiac masses, such as: fibroma, rhabdomyoma, myxoma, teratoma, and hemangioma³. In these tumors, the echogenic mass (single or multiple) is more localized and not around the fetal heart, as in our case.

Pulmonary malformations (adenomatoid malformation, and sequestrum) must also be included in the differential diagnosis; an intrapericardial extralobar sequestration, appearing as a solid mass, has been described in the pericardium after birth⁴.

Diaphragmatic hernia was also possible, but the continuity of the diaphragm was confirmed by sonograms. In the other known causes of fetal pericardial effusions (Rh disease, cardiac failure, hypoalbuminemia, immune diseases, renal dysplasia, pericarditis, other causes of hydrops, and normal transitory pericardial effusion of the second half of gestation), the fluid accumulation appeared always hypoechoic^5,6.

Prognosis

It depends upon associated anomalies and upon cardiac function. The prognosis of nonimmune fetal hydrops is very poor with a perinatal mortality rate between 70 to 90 percent¹.

Management

Sonography must search for signs of heart failure. When the diagnosis of a pericardial hemorrhagic effusion with hydrops is made before viability, the option of pregnancy termination should be offered.

References

1. Romero R, Pilu G, Jeanty P et al.: Nonimmune hydrops fetalis. In Prenatal diagnosis of congenital anomalies, East Norwalk, CT: Appleton & Lange, 1988; pp414-426

2. Swischuk LE: Pericardial abnormalities. In Imaging of the newborn, infant, and young child. Baltimore, Williams & Wilkins, 3rd edition, 1989; 334-338

3. Malisch TW, Jeanty P: Cardiac fibroma. The Fetus. 1991,p 2746-1

4. Levi A, Findler M, Dolfin T, et al.: Intrapericardial extralobar pulmonary sequestration in a neonate. Chest. 1990; 98:1014-1015

 5. Shenker L, Reed KL, Anderson CF, et al.: Fetal pericardial effusion. Am J Obstet Gynecol. 1989; 160:1505-1507

6. Jeanty P, Romero R, Hobbins JC: Fetal pericardial fluid: a normal finding of the second half of gestation. Am J Obstet Gynecol. 1984; 5:529-532