Case Reports in Obstetrics and Gynecology
Volume 2015 (2015), Article ID 154690, 11 pages
http://dx.doi.org/10.1155/2015/154690

Case Report

Prenatal Diagnosis of Cardiac Diverticulum with Pericardial Effusion in the First Trimester of Pregnancy with Resolution after Early Pericardiocentesis

Raquel Garcia Rodriguez, Azahara Rodriguez Guedes, Raquel Garcia Delgado, Lourdes Roldan Gutierrez, Margarita Medina Castellano, and Jose Angel Garcia Hernandez

Prenatal Diagnosis and Fetal Therapy Unit, Department of Obstetrics and Gynecology, University Maternity Hospital of Canaries, Las Palmas, 35004 Gran Canaria, Spain

Received 19 May 2015; Revised 6 September 2015; Accepted 13 September 2015

Academic Editor: Babatunde A. Gbolade

Copyright © 2015 Raquel Garcia Rodriguez et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract.

Cardiac diverticulum is a rare anomaly, which may present in association with pericardial effusion. Only few cases diagnosed during fetal life have been published and only in 12 cases pericardiocentesis was made with good postnatal outcomes in 83% of the cases. In the first trimester of pregnancy only 6 cases were reported. We described the largest series of cases published. We describe a case of cardiac diverticulum complicated with pericardial effusion during the first trimester of pregnancy and resolved by intrauterine pericardiocentesis at 17 weeks of pregnancy. We made a systematic review of the literature with the cases reported of cardiac diverticulum, management, and outcomes.

1. Introduction

Cardiac diverticulum is a rare anomaly, which may present in association with pericardial effusion and may produce complications such as pulmonary hypoplasia or hydrops fetalis. Only a few cases diagnosed during fetal life have been published and 10 cases during the first trimester of pregnancy.

We describe a case of cardiac diverticulum complicated with pericardial effusion, which was diagnosed at 14 weeks of pregnancy and resolved by intrauterine pericardiocentesis at 17 weeks of pregnancy.

We review the ultrasound findings, management, and outcomes in 34 cases published in the literature, 21 of which were associated with pericardial effusion, and in 12 cases a pericardiocentesis was performed. In this group of pericardiocentesis, 10 cases have good postnatal outcomes.

In light of the good neonatal outcome of this procedure, we propose that early pericardiocentesis should be considered as a therapeutic option in these cases that do not resolve spontaneously.

2. Case Report

A 22-year-old sub-Saharan woman, gravida 2 para 1, was referred to the Unit of Prenatal Diagnosis and Fetal Therapy at 12 + 4 weeks of gestation for a fetal hydrothorax. The ultrasonographic study revealed the presence of severe pericardial effusion. The first trimester screening showed 2.9 mm nuchal translucency, reversed ductus venosus a-wave, absent nasal bone, and absence of tricuspid regurgitation. All other anatomical and echocardiographic findings were normal. Biochemical markers were β-hCG 0.14 MoM and PAPP-A 0.72 MoM. The screening results indicated high risk of chromosomopathy. Therefore, a chorionic villus samplingbiopsy study was performed, which revealed a normal 46 XY karyotype. On week 14 of pregnancy, pericardial effusion was still observable. Furthermore, a two-millimeter anechoic image was found on the heart apex, with a narrow base and expanding from the ventricular wall into the pericardium. Blood flow could be observed through this structure with color Doppler sonography. On this basis, the diagnosis of a cardiac diverticulum was made (Figure 1). The rest of the echocardiography was normal.

Figure 1: Transversal view of the thorax during the 14th week of pregnancy, where severe pericardial effusion (thick arrow) and cardiac diverticulum in the ventricular apex (thin arrow) can be observed.

Serial ultrasound studies were conducted until week 17 of pregnancy (Figure 2). Given that the pericardial effusion persisted, pericardiocentesis with a 20-gauge needle (like amniocentesis) was performed and a clear serum-like fluid was removed. After pericardiocentesis, both lungs expanded and only a small amount of residual fluid remained. Results of the infections study of the amniotic fluid (Toxoplasma, cytomegalovirus, rubella, herpes virus, and parvovirus B19) were negative. A week after pericardiocentesis, no new pericardial effusion was detected. The clinical picture remained stable until week 25, when cardiomegaly and thickening of the ventricular walls were observed, thus leading to a diagnosis of hypertrophic myocardiopathy. Ultrasound studies showed normal ventricular function without signs of cardiac failure. On week 40, a boy was born in eutocic delivery, with 3150-gram body weight, 9/10 Apgar score, and 7.29 umbilical artery pH. The newborn was admitted to hospital for cardiological study. Subsequent serial examinations ruled out hypertrophic myocardiopathy, although they showed residual pericardial effusion, an apical cardiac diverticulum, persistent small ductus venosus, and permeable oval foramen. At present, the child is four years old, remains asymptomatic, and is on a prophylactic treatment with acetylsalicylic acid. He is being monitored in annual follow-up visits to the Service of Pediatric Cardiology.

Figure 2: Transversal view of the thorax during the 16th week of pregnancy. Severe pericardial effusion (thick arrow) and cardiac diverticulum in the ventricular apex that connects with the ventricle (thin arrow) can be observed.

3. Discussion

A cardiac diverticulum is a protrusion located on the ventricular wall, in close communication with it. This rare entity has been scarcely reported in the literature. Prenatal diagnosis may be difficult when it appears isolated [1]. Since such formations are closely communicated with the heart ventriculum, bidirectional blood flow may be observed through them with color or pulsed Doppler sonography [1].

Two types of diverticula are known: apical and nonapical [2, 3]; their characteristics are shown in Table 1. Apical diverticulum of the left ventriculum may be of three different types: apical isolated diverticula, which are not associated with other malformations; multiple diverticula, which are located on the diaphragmatic or anterior surface of the ventriculum; and large apical diverticula, which are associated with midline thoracoabdominal malformations or with the Pentalogy of Cantrell [4]. Although their etiology is not known, they seem to be caused by local weakening of the ventricular wall, which may in turn be due to possible embryogenesis defects [5], secondary to infections, or caused by local ischemia resulting from coronary anomalies, such as stenosis, hypoplasia, intimal proliferation, and thrombosis [6–8]. Differential diagnosis should include aneurisms (Table 1) [6, 9–12], myocardiopathy, Ebstein’s anomaly, and auriculoventricular regurgitation [9].

Table 1: Differential diagnosis of the different types of diverticulum and aneurism.

Isolated apical diverticulum
Nonapical diverticulum
Aneurism

Etiopathogeny
Embryogenesis defect
Congenital or acquired focal defect in the muscle wall (viral infection, injury to coronary artery, etc.)
Congenital or acquired focal defect in the muscle wall (viral infection, injury to coronary artery, etc.)

Implantation base on ventriculum
Narrow base
Narrow base
Wide base

Size
Small
Small
Large

Development during pregnancy
Constant size throughout pregnancy
Constant size throughout pregnancy
Enlarging with gestational age

Histology
Myocardium in walls; usually presenting the three layers (myocardium, pericardium, and endocardium)
Myocardium in walls, usually presenting the three layers (myocardium, pericardium, and endocardium)
Myocardium disruption, usually presenting thin myocardium and fibrous tissue

Kinesis
Normal
Normal
Akinetic, hypokinetic, and dyskinetic

Contractility
Contractility in synchrony with heart’s rhythm
Contractility in synchrony with heart’s rhythm
Contractility paradoxical with heart’s rhythm

Complications
Depending on the associated anomalies
Usually not occurring
Arrhythmia, thromboembolism, heart failure, and rupture

Prognosis
Good
Good
Bad

We made a systematic review of the literature with PubMed and Embase database search in English, French, or German which was performed without any restriction of publication date or journal, using the following key words: fetal cardiac diverticulum. The last search was updated in December 2012.

For each included case we focused on the following criteria: time of diagnosis, pericardial effusion association, management, intrauterine evolution, associated complications, and time of delivery fetal outcome (intrauterine death/stillbirth, death after delivery, and survival). We excluded the cases of cardiac aneurysm. After reviewing 27 articles, 34 cases published between 1990 and 2012 met the above criteria in which cases of fetal cardiac diverticulum were presented (Table 2) with our case included.

Table 2: Description of the cases of cardiac diverticulum reported in the literature.

Author
GA di
Size
Sex
Location
Karyotype
Associated anomalies
Intervention
Prenatal progression
Neonatal
Follow-up

1
Kitchiner et al. (1990) [13]
33
—
Female
Apex VI
—
Cardiomegaly
No
Stable
Vaginal delivery 40 w; cardiomegaly, tachypnea, heart murmur, muscular IVC, and mild mitral regurgitation
Asymptomatic at 3.5 months of life

2
Hornberger et al. (1994) [9]
31
—
—
Lateral wall below tricuspid valve (RV)
—
—
No
Stable
Vaginal delivery at term
Asymptomatic at 12 months of life

3
Carles et al. (1995) [24]
13
—
Male
Apex LV
—
Pericardial effusion
TOP 14 w
—
—
—

4
Cesko et al. (1998) [25]
17
—
Male
Apex RV
46XY
Pericardial effusion
TOP 22 w
Stable
—
—

5
Cavallé-Garrido et al. (1997) [6]
20
Large
Female
Lateral wall below mitral valve (LV)
Trisomy 18
Ventricular septal defect, hydrops
No
Fetal death 26 w
—
—

6
Cavallé-Garrido et al. (1997) [6]
19
Small
Female
Apex RV
—
No
No
Stable spontaneous resolution at 34 w
Asymptomatic
Asymptomatic at 22 months of life

7
Cavallé-Garrido et al. (1997) [6]
19
Small
—
Apex RV
—
Pericardial effusion
PC 20 w
Stable
Asymptomatic
Asymptomatic at 12 months of life

8
Cavallé-Garrido et al. (1997) [6]
36
Small
Male
Lateral wall below tricuspid valve (RV)
—
Pericardial effusion

Asymptomatic at 18 months of life

9
Johnson et al. (1996) [16]
19
3 mm
Female
Apex RV
46XX
Pericardial effusion
PC 20 w
No relapse after PC, no growth
Eutocic delivery 41 w; weight 3700 grams; asymptomatic
Asymptomatic at 16 months of life

10
Brachlow et al. (2006) [23]
32
—
—
Apex LV
—
Cardiomegaly
No
Stable
—
Asymptomatic at 6 months of life

11
Bernasconi et al. (2004) [26]
22
10 × 5 mm
Male
LV lateral wall below mitral valve
46XY
Pericardial effusion
PC 22 w
—
Fetal death 26 w, probably due to diverticulum rupture
—

12
McAuliffe et al. (2004) [27]
13
4 × 6 mm
Male
Apex RV
46XY
First trimester NT 4.2 mm
Pericardial effusion
PC 16 w
Resolution of the effusion; CD stable
Eutocic delivery 38 w; weight 3070 grams; asymptomatic
Asymptomatic at 10 months of life

13
McAuliffe et al. (2004) [27]
13
4 × 3 mm
Male
Apex RV
46XY
First trimester NT 2 mm
Pericardial effusion
PC 14 w
Resolution of the effusion; CD stable
Eutocic delivery 38 w; weight 3150 grams; asymptomatic
Asymptomatic at 8 months of life

14
Prefumo et al. (2005) [1]
14
5 × 5
Male
Apex RV
46XY
First trimester NT 3.7 mm; pericardial effusion, ascites, and skin edema
PC 16 w
Resolution of the effusion and hydrops; CD stable; mild cardiomegaly
Vaginal full-term eutocic delivery; asymptomatic
Asymptomatic at 22 months of life

15
Prefumo et al. (2005) [1]
12
1 mm
—
Apex RV
—
First trimester NT 1.2 mm Pericardial effusion
No
Spontaneous resolution of PE with 21 w; CD stable
Full-term eutocic delivery, asymptomatic
Asymptomatic at 17 months of life

16
Gardiner et al. (2005) [19]
14
2-3 mm
—
Apex RV
Normal
Pericardial effusion
PC 14 w
Resolution of the effusion and hydrops; CD collapsed
Asymptomatic at birth
—

17
Gardiner et al. (2005) [19]
14
2-3 mm
—
Apex RV
Normal
Pericardial effusion
TOP
—
—
—

18
Del Río et al. (2005) [18]
13
5 × 5
Female
Apex RV
46XX
Pericardial effusion, septal defect AV
No
Spontaneous resolution at 28 w
Eutocic delivery 40 w; weight 3400 grams, asymptomatic at birth
Correction of septal defect at 3 months of life, resection of diverticulum; asymptomatic at 8 months of life

19
Wax et al. (2007) [14]
20
6 × 9 mm
Male
Junction base RV-infundibulum
—
No
No
Stable
Full-term eutocic delivery; weight 3689 grams; asymptomatic; small permeable FO
Asymptomatic at 18 months of life

20
Koshiishi et al. (2007) [17]
24
7 × 10 mm
—
Lateral wall below tricuspid valve (RV)
—
Mild pericardial effusion; MC pregnancy with laser intervention for TTTS at week 20 where donor fetus died
No
Stable
Prenatal fetal death at 29 w
—

21
Pradhan et al. (2007) [28]
28
—
—
Apex LV
—
Fetal arrhythmia
Hydrops fetalis
Medical treatment (digoxin)
—
Vaginal delivery 40 w
Asymptomatic at 12 months of life

22
Barberato et al. (2009) [29]
16
5 × 5.7 mm
—
Apex LV
—
Mild pericardial effusion
PC 20 w
Discrete enlargement of PE with normal heart function
Prenatal fetal death 37 w
—

23
Barberato et al. (2009) [29]
30
12 × 13 mm
—
Mitral subvalvular
—
LV dilatation and reduced systolic function
No
Stable
—
Asymptomatic at 6 months of life

24
Davidson et al. (2006) [15]
20
—
—
Apex RV
—
Pericardial effusion
No
Spontaneous resolution
—
Surgical treatment

25
Williams  et al.(2009) [3]
21
5 × 5.5 mm
Male
RV
—
Pericardial effusion
PC 24 w
Mild tricuspid regurgitation at 31; CD stable
Full-term delivery
Asymptomatic at a year of life

26
Perlitz et al. (2009) [30]
22
7 × 4 mm
Male
RV lateral wall
—
No
No
Stable, CD growth up to 9 × 9 mm
Eutocic delivery week 40; weight 4010 grams; asymptomatic at birth
Asymptomatic at a year of life

27
Menahem (2010) [31]
19
—
—
Apex LV
—
Pericardial effusion
—
No controls performed
Full-term live birth
Asymptomatic at 10 months of life

28
Carrard et al. (2010) [32]
13
2.6 × 2.9 mm
Male
RV lateral wall
46XY
First trimester NT 2.2 mm
Pericardial effusion
PC 17 w
Resolution after PC; CD collapsed at 26 w
Eutocic delivery 40 w, 2780 grams
Asymptomatic at 11 months of life

29
Abi-Nader  et al. (2009) [2]
22
3-4 mm
Male
RV
46XY
Pericardial effusion
No
Resolution at 32-33 w
PROM 34 w; Intubation due to prematurity; caesarean section; weight 2460 gr; 2 muscle IVCs
Asymptomatic at 14 months of life

30
Abi-Nader et al. (2009) [2]
21
11 × 15 mm
Male
RV lateral wall below tricuspid valve
—
Isolated
—
—
Eutocic delivery; weight 2780 gr; asymptomatic at birth
Asymptomatic at 16 months of life

31
Abi-Nader et al. (2009) [2]
25
26 × 16 mm (37 s)
Male
RV
—
Arrhythmia and reduced systolic function
Induced delivery
—
Caesarean section 38 + 5 w; weight 3270 grams; mild reduction of systolic function and premature ventricular contractions at birth
Asymptomatic at 3 years of life, on prophylactic treatment with acetyl salicylic acid

32
Williams et al. (2009) [3]
17

—
Apex LV
Normal
Mesocardia, perimembranous IVC
No
Stable
Full-term live birth
Asymptomatic at 2 years of life

33
Paoletti  and Robertson (2012) [20]
21
1.6 × 0.4 mm
—
Apex LV
Normal
Defect on thoracoabdominal midline
TOP
—
—
—

34
Nam et al. (2010) [21]
31
12 mm (postnatal)

RV lateral wall below tricuspid valve
—
—
No
Ventricular septal defect
Full-term live birth; asymptomatic at birth; symptoms at 45 days of life: closure of septal defect at 3 months of life
Asymptomatic at 10 months of life

35
Our case
14
2 mm
Male
Apex RV
46XY
Pericardial effusion
PC 17 w
PE resolution after treatment; CD stable; moderate cardiomegaly; normal heart function
Full-term live birth; spontaneous eutocic delivery 40 + 1 w; weight 3150 grams
Asymptomatic at 4 years of life

GA di: gestational age at diagnosis; RV: right ventriculum, LV: left ventriculum; w: weeks of pregnancy; TOP: termination of pregnancy; PC: pericardiocentesis; CD: cardiac diverticulum; IVC: interventricular communication PE: pericardial effusion; PROM: premature rupture of membranes; NT: nuchal translucency.

Diagnosis was made during the pathological examination after death. Diagnosis of the ventricular septal defect was made after birth.

It was most frequently diagnosed during the second trimester of pregnancy (51%). During the first trimester, 29% of cases were diagnosed; three of them presented increased nuchal translucency. This entity affects male fetuses more than female ones (3 : 1); its most frequent location is on the right ventriculum (70%) and the most frequently involved area is the heart apex (57%). Ultrasonographic findings associated with diverticula include pericardial effusion, cardiomegaly, septal defects and arrhythmia with fetal death before delivery, and hydrops [6, 13, 14]. Pericardial effusion is the most frequently associated finding (63%) and should be considered an indirect sign of the presence of cardiac diverticula. Although the etiology of effusion is not known, it has been proposed to result from the diverticulum rubbing the pericardial walls or from heart failure. Thus, the observation of pericardial effusion makes it necessary to examine the cardiac function [1, 6, 15].

A problem associated with pericardial effusion is that the resulting compression may produce heart failure and pulmonary hypoplasia. The management of such cases (Table 3) usually varies from performing pericardiocentesis to adopting an expectant approach. In a published series of 22 cases, pericardiocentesis was performed in 12 (56%) fetuses of 14 to 25 weeks’ gestational age, with an outcome of 10 cases that progressed favorably (83%) and 2 intrauterine deaths (17%) [16, 17]. In the cases of fetal death, the pericardial fluid was blood. In the same series, 7 cases were managed with an expectant approach (32%). Five of them showed spontaneous resolution (71%) and 2 resulted in intrauterine death (29%): one of them, which occurred on week 26, was associated with trisomy 18 and the other, which occurred on week 29, was associated with treated twin-to-twin transfusion syndrome and death of one of the twins after treatment [6, 18].

Table 3: Management and outcomes of the cases with cardiac diverticulum and pericardial effusion.

Reference
GA PE
GA Di
Loc.
Size (mm)
Intervention
PE findings
Prenatal progression
Postnatal progression

1
Carles et al. [24]
13
—
Apex LV
—
TOP 14 w
—
—
—

2
Cesko et al. [25]
17
AP
Apex RV
3 mm
TOP 22 w
—
—
—

3
McAuliffe et al. [27]
14
14
Apex RV
2-3 mm
TOP
—
—
—

4
Cavallé-Garrido et al. [6]
19
—
RV
3 mm
No
—
Spontaneous resolution at 34 w
Asymptomatic at 22 months

5
Cavallé-Garrido et al. [6]
20
—
LV lateral wall below mitral valve
Large
No
—
Prenatal fetal death at 26 w, trisomy 18
—

6
Prefumo et al. [1]
12
12
Apex LV
1 mm
No
—
Spontaneous resolution, effusion disappeared at 14 weeks; CD was not visible on ultrasound examination from week 21
Asymptomatic at birth effusion or diverticulum not visible; asymptomatic at 17 months’ follow-up

7
McAuliffe et al. [27]
13
13
Apex RV
5 × 5 mm
No
—
Spontaneous resolution; CD did not grow Perimembranous IVC
IVC and IAC (postnatal); asymptomatic up to 3 months of age; surgical treatment; asymptomatic at 8 months of age

8
Pradhan et al. [28]
20
20
Apex RV
—
No
—
Spontaneous resolution CD did not grow
Surgical treatment at birth

9
McAuliffe et al. [27]
21
24
RV lateral wall
7 × 10 mm
No
—
Fetal death on week 29
—

10
Perlitz et al. [30]
19
19
Apex LV
—
No
—
No control performed
Full-term live birth; asymptomatic at 10 months of age; heart murmur; no treatment

11
Cavallé-Garrido et al. [6]
19
—
Apex RV
—
PC 20 w
—
No PE relapse, CD did not grow
Full-term live birth; asymptomatic at 12 months of age

12
Carles et al. [24]
19
19
Apex RV
3 mm
PC 20 w
7 cm³ yellow fluid, 20 gr/L proteins (transudate), acellular
No PE relapse, CD did not grow
Full-term live birth; asymptomatic at 16 months of age; no treatment

13
Cesko et al. [25]
22
AP
Pared lateral LV
10 × 5 mm
PC 25 w
25 mL old blood fluid
Intrauterine fetal death at 26 weeks (CD rupture)
—

14
Brachlow et al. [23]
13
13
Apex RV
4 × 6 mm
PC 16 w
3 mL serohematic fluid, 18 gr/L proteins (transudate), lymphocytes, and mesothelial cells
No PE relapse or enlarging; CD was not visible on week 37
Full-term live birth; asymptomatic at 10 months of age; no treatment

15
Brachlow et al. [23]
13
13
Apex RV
4 × 3 mm
PC 14 w
0.8 mL serohematic fluid, 15 gr/L proteins (transudate)
No PE relapse; CD did not grow
Full-term live birth; asymptomatic at 8 months of age; no treatment

16
Prefumo et al. [1]
14
14
Apex RV
5 × 5 mm
PC 16 w
5 mL clear fluid
No PE relapse; CD did not grow; mild cardiomegaly
Full-term live birth; asymptomatic at 22 months of age; no treatment

17
Gradiner et al. [19]
14
14
Apex RV
2-3 mm
PC 14 w
2 mL yellow fluid
No PE relapse; CD did not grow
Full-term live birth; asymptomatic; no treatment

18
Carrard et al. [32]
13
15
Apex RV
2.6 × 2.9
PC 17 w
4 mL clear fluid, 21 g/L proteins (transudate)
No PE relapse; diverticulum was not visible from week 26 on
Full-term live birth; asymptomatic at 11 months of age; no treatment

19
Williams et al. [3]
21
21
Apex RV
5 × 4.5
PC 24 w
Yellow fluid 10 mL, 15.4 g/L proteins (transudate), lymphocytes
Complete resolution one week after PC CD did not grow
Full-term live birth; asymptomatic at one year of age; no treatment

20
Barberato et al. [29]
16
16
—
—
PC 20 w
Blood-stained fluid
Moderate growth of PE size as compared with postpuncture effusion; expectant approach Intrauterine fetal death on week 37
—

21
Abi-Nader et al. [2]
12
22
Apex RV

PC 18 w
—
Relapse one week later and subsequent spontaneous resolution on weeks 32-33
—

22
Our case
12
14
Apex RV
2 mm
PC 17 w
Clear yellow fluid, acellular, transudate
No PE relapse; CD did not grow; moderate cardiomegaly
Full-term live birth; asymptomatic at birth; treatment with ASA; asymptomatic at 4 years of age

GA PE: gestational age at pericardial effusion; GA di: gestational age at diverticulum diagnosis; RV: right ventriculum, LV: left ventriculum; w: weeks of pregnancy; PC: pericardiocentesis; CD: cardiac diverticulum; IVC: interventricular communication; PE: pericardial effusion.

Our review is the largest series published in the literature with 34 cases of fetal cardiac diverticula. If we exclude the cases of termination of pregnancy, chromosomal abnormalities, and pregnancy complications (twin-to-twin transfusion syndrome), the cardiac diverticulum is a benign condition with good postnatal outcomes, with a mortality of 0.6%. In the cases with pericardial effusion, a pericardiocentesis, performed from 16 weeks of gestation with a 20-gauge needle, seems to be a safe procedure with good outcomes in 83% of cases. The evolution of the pericardial effusion and the diverticula (spontaneous resolution, stability, or progression) is not known because there are few cases published, but the risk of cardiac insufficiency, hydrops fetalis, or pulmonary hypoplasia could be avoided with this procedure.

In our case we made an early diagnosis and treatment with pericardiocentesis with resolution of the pericardial effusion with an excellent outcome. Only 10 cases (29%) were reported during the 11–14 weeks’ scan and only 6 cases (17%) with pericardial effusion associated during this period.

Cardiac diverticula are rarely associated with chromosomopathies especially when other malformations are not present [5]. Cardiac abnormalities usually associated with diverticula include ventricular or auricular septal defects, permeable oval foramen, tricuspid atresia, tetralogy of Fallot, persistent left superior vena cava, pulmonary artery hypoplasia, and coarctation of the aorta [5]; cardiomegaly is the most frequently associated complication in the third trimester, like in our case [6, 19]. Usually associated extracardiac malformations include midline thoracoabdominal defects (omphalocele, ectopia cordis, Pentalogy of Cantrell, etc.) [2, 5, 8, 20–22].

The prognosis of this entity is generally good, although the outcome largely depends on the size and location of associated anomalies. Cases of rupture, both pre- and postnatal, arrhythmia, fetal death, heart failure, and coronary insufficiency have been described [9, 16, 18, 21, 23]. In these patients, serial control examinations are necessary to detect possible complications. In general, postnatal progression is good and surgery is not necessary in asymptomatic cases [19].

In conclusion, a cardiac diverticulum is a rare entity. The most frequently associated complication is pericardial effusion, which may result in cardiac decompensation, hydrops fetalis, or pulmonary hypoplasia. Although spontaneous resolution of effusion has been reported, pericardiocentesis is a safe and effective technique, which may be used to reduce the risk of secondary disorders in selected cases. Nowadays there are few cases published and the evidence is poor to establish the appropriated management.

Conflict of Interests

The authors declare no conflict of interests.

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