European Journal of Case Reports in Internal Medicine

 

The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM). The journal wants to promote the practice of internal medicine in Europe. Its aim is to provide a forum to internal medicine doctors.
EJCRIM welcomes papers describing unusual or complex cases and case series that an internist may encounter in everyday practice. Case series are also welcomed as long as they demonstrate the appropiateness of a therapeutical approach or unusual manifestation of a disease.
The journal would also consider brief reasoned reports of issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to scientific meetings of European societies of Internal Medicine.

EJCRIM is peer-reviewed with single-blind review and freely accessible to all.

 
Itala Marina Baldini, Cristina Cocino, Sonia Seghezzi, Maria Domenica Cappellini
Hugo M Oliveira, Claudia Pereira, Ermelinda Santos-Silva, Jorge Pinto-Basto, José R Vizcaíno, Helena Pessegueiro-Miranda
Lidor Akavia, Ilan Krauze
Filipa Brás Monteiro, Sofia Cristóvão Ferreira, Rita Mendes
Carolina Oliveira Ourique, Inês Chora, Susana Guimarães, Sara Júlio, Paula Vaz-Marques
Annamaria Gesualdo, Rossana Tamburrano, Antonia Gentile, Antonio Giannini, Giuseppe Palasciano, Vincenzo Ostilio Palmieri
Angela Daher, Fady Haddad
Ángel Torralba-Morón, Juan Ortiz-Imedio, Montserrat Morales-Conejo, Juan Ruiz-Morales, Juan-Manuel Guerra-Vales

Sometimes Surgery is the Only Way to Make a Diagnosis and Treat the Patient: A Case of Congenital Partial Absence of Pericardium

Phivos C Symeonides
Published: 06/04/2017

ABSTRACT

Introduction: A 47-year-old Caucasian woman with a past medical history of multiple ablative procedures for supraventricular arrhythmias and pacemaker implantation presented with increasing shortness of breath. The initial working diagnosis of the team treating her was ablation-induced pulmonary stenosis, especially after the recording of increased flow velocities through the right lower pulmonary vein.
Case presentation: The patient was alert and oriented, but obviously dyspnoeic. The vital signs were normal. The physical examination revealed a soft cardiac systolic murmur and the lungs were clear on auscultation. The electrocardiogram showed a pacemaker rhythm. The echocardiogram showed borderline normal global systolic function of the left ventricle and severe mitral regurgitation. The transoesophageal echocardiogram confirmed the above findings and revealed increased velocities through the right lower pulmonary vein. The working diagnosis of ablation-induced pulmonary stenosis was reinforced by the cardiac CT angiography. The patient was subsequently referred for surgical intervention. The intra-operative findings were both unexpected and impressive: congenital partial absence of the pericardium was responsible for herniation of the right chambers into the pleural space. Mitral regurgitation was attributed to failure of coaptation due to the very short surface of the leaflets. Extensive external fibrosis around the pulmonary veins caused the pulmonary vein stenosis.
Conclusion: The final diagnosis of a partial pericardial defect causing torsion and distortion of the heart chambers was made only at surgery. The consistent finding of pulmonary vein stenosis in the non-invasive modalities and the past medical history of ablations initially misleadingly led us to the assumption that they were related.

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