A Rare Case of Gerbode Defect and Review of Literature
Anirudh Kumar Paidi,
Palli Venkata Naresh Kumar,
Ravikanth Vanguri,
Rajasekhar Varada,
Madhav Rao Midhe,
Ravi Kiran Mamidala
Issue:
Volume 5, Issue 6, November 2019
Pages:
76-79
Received:
26 October 2019
Accepted:
19 November 2019
Published:
25 November 2019
Abstract: A Gerbode defect is a very rare type of congenital anomaly with a direct communication between left ventricle and right atrium leading to Left to Right shunt. This defect was first reported by Meyer in 1857. First successful closure was done by Kerby et al using hypothermia and inflow occlusion technique. It may present as a direct or indirect type. With improvements in nomenclature and taxonomy that expanded the classification until the current modifications were in place that accounted for defect type and position with respect to the Tricuspid Valve. Now, it is broadly described as Supravalvular type and infravalvular type. The etiology is typically congenital with irregularities emerging by perforation of anterior intraventricular septum, malformation of leaflets, or widening of the commissural space. These embryological deviations subsequently permit an abnormal communication that begins the physiological processes leading to the pathology. Shunt depends on the size of the defect and PVR. Shunt flow begins inutero because of the obligatory difference in the systolic pressure between LV and RA. RA accepts shunt with less or no elevation of pressures due to distensibility. We report a 8 year old female child who presented with dyspnea on exertion and recurrent respiratory infections whose echocardiography confirmed a direct type (supravalvular) Gerbode defect. This was confirmed by cath study and during surgery. She underwent successful suture closure of the shunt using a Goretex patch. This is a case of direct type Gerbode defect doing well after surgery.
Abstract: A Gerbode defect is a very rare type of congenital anomaly with a direct communication between left ventricle and right atrium leading to Left to Right shunt. This defect was first reported by Meyer in 1857. First successful closure was done by Kerby et al using hypothermia and inflow occlusion technique. It may present as a direct or indirect type...
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Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult
Rafael Meza,
John Araujo,
Alejandro Escobar,
Alejandra Echeverri,
Juan Turizo,
Susana Cardona
Issue:
Volume 5, Issue 6, November 2019
Pages:
80-83
Received:
30 November 2019
Accepted:
19 December 2019
Published:
7 January 2020
Abstract: Scimitar syndrome is an infrequent congenital malformation. It is a partial anomalous pulmonary venous drainage of the right lung into the inferior vena cava. Up to 25% of cases are associated with other heart diseases. The incidence in adults is reported to be 0.5-0.7%; classically, the adult form does not have pulmonary arterial hypertension, and may be unnoticed and asymptomatic for many years. It often presents after the second decade of life with nonspecific symptoms. Without surgical repair, clinical worsening, increased pulmonary pressure and sustained pulmonary arterial hypertension ultimately occur, when the syndrome is associated with other congenital heart diseases. We present the case of a 38-year-old woman diagnosed with scimitar syndrome associated with a large superior sinus venosus atrial septal defect, who underwent successful surgical repair using a lateral pericardial tunnel technique to redirect right pulmonary venous flow to the left atrium through a left atriotomy. This repair has shown good medium-term results. Possible complications such as tunnel thrombosis and stenosis will need to be explored in future assessments, and follow up must be lifelong.
Abstract: Scimitar syndrome is an infrequent congenital malformation. It is a partial anomalous pulmonary venous drainage of the right lung into the inferior vena cava. Up to 25% of cases are associated with other heart diseases. The incidence in adults is reported to be 0.5-0.7%; classically, the adult form does not have pulmonary arterial hypertension, and...
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