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Sumario Vol. 42 - Nº 3 Julio - Septiembre 2013

Fibrous Right Ventricular Diverticulum and Pericardial Effusion in an Adult Patient. A Rare Finding

Javier Courtis (1), Ricardo Liendo (2),
Ernesto Juaneda (3), José Tibaldi (4)

Departamento de Imágenes Cardiovasculares
y Cardiología Intervencionista Instituto Oulton (1,3,4),
Departamento de Cirugía Cardiovascular (2) Sanatorio Allende.
Instituto Oulton. Av. Vélez Sarsfield 562 (5000) Córdoba, Argentina
E mail

Recibido 02-Julio-2013 – ACEPTADO después de revisión el 17-AGOSTO-2013.

The authors declare not having a conflict of interest.
Rev Fed Arg Cardiol. 2013; 42(3): 215-218


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RESUMEN

Un divertículo ventricular es una enfermedad congénita de la pared cardíaca, raramente diagnosticada por primera vez en la vida adulta. Se describe la resección quirúrgica exitosa de un divertículo cardíaco fibroso del ventrículo derecho acompañado de derrame pericárdico en una paciente adulta.

Palabras clave: Divertículo ventricular. Derrame pericárdico. Cirugía cardíaca
SUMMARY

A ventricular diverticulum is a congenital disorder of the heart wall, rarely first diagnosed in adulthood. We describe the successful surgical resection of a fibrous cardiac diverticulum of the right ventricle accompanied by pericardial effusion in an adult patient.

Key words: Ventricular diverticulum. Pericardial effusion. Cardiac surgery

 

 

 

INTRODUCTION
A ventricular diverticulum (VD) is a protrusion located in the ventricular wall with a close connection to the cardiac chambers. In the adult population, the incidence observed was 0.4% in a series of autopsies of patients with cardiac death and 0.04% in a large retrospective study with echo. It is more frequent in the left ventricle (LV) and the isolated VD of the right ventricle (RV) is a finding even more rare [1].

 

CLINICAL CASE
A 30-year-old woman with history of 3 months of frequent palpitations was sent to our center for the evaluation of a possible heart disease. Her pathological history was irrelevant and the family history was negative for cardiovascular diseases and sudden cardiac death. The physical exploration was normal, the results of the lab tests were normal, and serology for Chagas disease was negative. Chest X-ray showed a cardiac water bottle shape and in the 12-lead ECG, sinus rhythm, low voltage in the limb leads and diffuse alterations in ventricular repolarization were observed. Transthoracic echo (TTE) revealed a saccular and hypoechoic image in the RV free wall; besides, it was rounded, with dyskinetic contractile movement and accompanied by moderate pericardial effusion with no hemodynamic involvement (Figure 1). Later, cardiac magnetic resonance (NMR) was conducted, which showed saccular and focal evagination of the RV free wall, with dyskinetic movement and a narrow neck. Late enhancement with Gd was not observed in the VD wall and at the pericardium level. Other relevant findings included moderate pericardial effusion with non-hemorrhagic aspect and dilated left pulmonary vein (Figure 2). To establish a more appropriate management, cardiac tomography was performed with a scanner of 64 lines of detectors. The results were similar to cardiac NMR and it was also confirmed that the patient presented a single left pulmonary vein (single left pulmonary vein confluence) and there was no obstructive CAD or vascular malformations associated (Figure 3). The patient underwent a surgical resection of the evagination by right lateral thoracotomy. During the operation, a 10 mm sac was identified in the RV free wall and pericardial effusion. The VD was resected, and a Dacron patch was applied. The ventricular muscle was closed and the pericardial liquid was drained (Figure 4). The pathological test revealed that all the evagination wall was made up of fibrous tissue and the adjacent pericardium showed chronic and mild inflammation. There was no fat infiltration or coagulation necrosis observed in the tissue studied (Figure 5). The final diagnosis was fibrous and isolated ventricular diverticulum in the RV free wall with an adjacent region of pericarditis and as a probable consequence of this chronic irritation, a moderate pericardial effusion. As associated malformation, the patient also presented a single left pulmonary vein. The post-operative period presented no complications and the patient was discharged, remaining asymptomatic for the 6 months after the surgery.

Figure 1. Chest X-ray shows cardiac shape with water-bottle aspect (A). In ECG (B), low voltage in the limb leads and diffuse ventricular repolarization alterations are observed. 2-D Echo (C) shows hypoechoic saccular image in the right ventricular free wall.

 

Figure 2. Cardiovascular magnetic resonance. In the SSFP-b cine sequence, RV free wall diverticulum and dilated left pulmonary vein are observed (A). During the first passage with Gd (B), the diverticular sac is completely filled with contrast, with no manifest late Gd enhancement in the diverticulum wall and the pericardium (C,D).

 

Figure 3. Multislice cardiac tomography. In the volumetric images, the diverticulum (A) and a single left pulmonary vein (B) are observed. In the axial view (C), the diverticular sac with a narrow neck is observed and the coronal view (D) shows moderate pericardial effusion (arrows).

 

Figure 4. Intraoperative. A 10 mm diverticulum is observed in the RV free wall (A), and its resection (B).

 

Figure 5. The pathological test with hematoxylin and eosin (A,B) and Masson’s thrichrome staining (C,D) show that the diverticulum wall is constituted by an area of fibrous tissue without fibroblasts, coagulation necrosis and the adjacent pericardium shows mild inflammation.

 

 

DISCUSSION
The etiology of VD is not so well known, and it is considered to be congenital if there is no history of pathological conditions that may have caused myocardial damage. It is commonly accompanied by other cardiac malformations, such as ventricular septal defect and tetralogy of Fallot. Specifically, a LV diverticulum may be part of the so-called Pentalogy of Cantrell, a rare syndrome that evolves with defects affecting the diaphragm, the abdominal wall, the pericardium, the heart and part of the sternum [2]. In literature, the size of VD described is variable, oscillating from very small ones (0.5 cm of diameter) to very large (8-9 cm). One of their main features is that they have a close connection with the ventricular cavities and they have a circular shape [3]. Although the most common locations of VD are the apical area of the left heart and perivalvular, they have been described in almost all the possible places in the ventricular walls [4]. According to the pathological findings, VD are classified into two types, muscular and fibrotic [5]. The muscular form, more frequent in children and the LV apex, tends to show contractile function synchronized with the ventricle (normokinesis) and is associated to other congenital heart disorders, particularly Cantrell’s syndrome. Histologically, it contains all the normal layers of the ventricular wall and presents a preserved myocardial architecture [6]. On the other hand, a fibrous diverticulum is more frequent in adults and it frequently shows contractile dysfunction (akinesis or dyskinesis). Histologically, it is essentially constituted by connective tissue, which is rich in reticulin and there are very few or no cardiac muscle fibers. These patients may be asymptomatic and often it is difficult to distinguish them from ventricular aneurysms. In general, the differentiation between both can only be made if there is an accurate knowledge on the cardiac history of the patient and the presence of associated anomalies, particularly CAD. Many authors have reported complications associated to VD (cardiac arrhythmias, sudden cardiac death, endocarditis, systemic embolisms, rupture); but the real incidence of the latter are unknown and most patients remain asymptomatic and without complications for all their lives, suggesting a benign clinical evolution [7]. The treatment of a VD has not been standardized and to this date, there are no large trials with long periods of observation to evaluate the best therapeutic option in this type of patients. Although surgical resection is the treatment of choice in symptomatic individuals, in asymptomatic ones it often represents a therapeutic dilemma [8]. Based on the data observed in literature, the decision about managing a patient with VD should adapt to the clinical and morphological characteristics of the sacculation, taking into account the associated anomalies and the possible complications in the short and long run.

CONCLUSIONS
The clinical case presented here illustrates about this rare nosological process, it guides us how to evaluate it, the results we will obtain from the different supplementary methods and it shows how the surgical resection of the diverticulum generated a favorable evolution in the patient, thus supporting the decision adopted by our team to treat her.

 

ACKNOWLEDGEMENT
To Angeles Ratti, B.S., for her help while preparing this manuscript.

 

REFERENCES

  1. Cianciulli TF, Gonzalez Colaso P, Saccheri MC, et al. Left ventricular diverticulum, a rare echocardiographic finding: two adult patients and review of the literature. Cardiol J 2009; 16 (1):76-81.
  2. Makkuni P, Kotler MN, Figueredo VM. Diverticular and aneurysmal structures of the left ventricle in adults: report of a case within the context of a literature review. Tex Heart Inst J 2010; 37 (6): 699-705.
  3. Teske DW, McGovern JJ, Allen HD. Congenital fibrous left ventricular diverticulum. Am Heart J 1993; 126: 1233-35.
  4. Ohlow MA. Congenital left ventricular aneurysms and diverticula: definition, pathophysiology, clinical relevance and treatment. Cardiology 2006; 106: 63-72.
  5. Wu JM, Yu CY. Isolated congenital left ventricular diverticulum. Pediatric Cardiol 1996; 17: 254-6.
  6. Mady C. Left ventricular diverticulum: analysis of two operated cases and review of the literature. Angiology 1982; 33: 280-6.
  7. Archbold RA, Robinson NM, Mills PG. Long-term follow-up of a true contractile left ventricular diverticulum. Am J Cardiol 1999; 83 (5): 810-3, A11.
  8. Srichai MB, Hecht EM, Kim DC, et al. Ventricular diverticula on cardiac CT: more common than previously thought. AJR Am J Roentgenol 2007;189 (1): 204-8.

Publication: September 2013

 
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