Anatomical basis for the treatment of agenesis of the left pericardium

Abstract

Summary.Three new cases of pericardial agenesis are reported: two cases of partial agenesis and one of total agenesis. All three cases were operated (resection of the left atrium).Following description of their cases, the authors provide an historical perspective of this disorder and analyse its frequency. A consideration of the embryology of the region explains why left sided agenesis is by far the more frequent. Finally, they discuss the appropriate diagnostic approach and suitable treatment, comparing total agenesis which is usually well tolerated with partial agenesis which exposes the patient to the risk of atrial herniation and occasionally sudden death. Bases anatomiques du traitement des ag6n6sies du p6ricarde gauche. A propos de trois observationsR6sum6. Trois nouvelles observations d'ag6n6sie du p6ricarde sont rapport6es: 2 cas d'ag6n6sie partielle, 1 cas d'ag6n~sie totale. Ces trois malades ont 6t6 op6-r6s (r6section de l'auricule gauche).Les auteurs envisagent ensuite l'historique et la fr6quence de cette anomalie, puis l'embryologie qui explique la plus grande fr6quence des aplasies du p6ricarde gauche. Enfin ils envisagent les circonstances diagnostiques et la conduite th6rapeutique, en opposant les formes globales, parfaitement tol6r6es, aux formes partielles (foramen p6ricardique), qui exposent au risque d'incarcSration de l'auricule gauche avec parfois mort subite.

Congenital diaphragmatic hernia with concurrent aplasia of the pericardium in a foal

“…In dogs, like in humans, the defects affecting the left side of the pericardium are more frequently observed [ 11 , 13 ]. Pericardial aplasia represents the partial defect, whereas agenesis the total congenital defect of the pericardium, which, based on anatomical classification are divided in total agenesis or unilateral agenesis of the left and right pericardium [ 39 ]. Sometimes the diaphragm and caudal pericardium share a common defect resulting in a peritoneopericardial hernia, a distinct form of diaphragmatic hernia previously described also in horses [ 40 ].…”

“…Its origin hypothetically was associated with the rotation of the heart into the left chest [ 47 ] due to the absence of the support role of the pericardium and increased tension on the anchoring structures of the heart [ 41 ]. Additionally, pectus excavatum [ 48 ], systolic heart murmurs on the pericardial defect [ 39 , 49 ], sinus bradycardia [ 47 ] and dyspnea [ 50 , 51 ] are occasionally observed. Although rarely reported, fatal syncope represents the most severe consequence of pericardial defect [ 52 , 53 ].…”

“…Hypothetically this cardiac dilatation was attributed to the absence of limiting effect of the pericardium which normally restrains the heart [ 55 ]. In humans and dogs, spontaneous cases of pericardial defects are rarely associated which cardiac deformities [ 11 , 13 , 39 , 49 ].…”