Mild Cyanosis due to Coexistence of Congenitally Corrected Transposition of the Great Arteries and Gerbode-Type Defect

Expert review Left ventricular to right atrial shunt (Gerbode defect): congenital versus acquired

“…Aetiologies of the LV-RA shunts were congenital in 32 (26.4%) cases [ 6 , 7 , 9 – 28 , 30 – 32 ], acquired in 88 (72.7%) cases [ 2 , 3 , 7 , 8 , 18 , 31 , 33 – 68 , 70 – 90 , 92 , 94 – 104 , 106 – 108 , 110 , 111 ] and unknown in 1 (0.8%) patient [ 112 ], respectively. A significant difference was noted in the percentages between congenital and acquired LV-RA shunts (χ 2 = 52.3, p < 0.0001).…”

“…The 11 misdiagnosed patients were initially mistaken as tricuspid regurgitation [ 6 , 22 ] and tricuspid regurgitation and pulmonary artery hypertension [ 25 , 83 , 84 ] in 3 (27.3%) patients each and rupture of Valsalva aneurysm into the right atrium [ 14 ], prosthetic mitral valve leak [ 103 ], mitral valve regurgitation with prosthetic mitral valve leak [ 92 ], atrial septal defect [ 47 ] and residual VSD [ 8 ] in 1 (9.1%) patient each. There were 45 TTE images leading to misdiagnosis, missed diagnosis, or inclusive diagnosis in 33 patients, whose correct diagnoses were established mostly by alternative diagnostic techniques, with TEE being the most frequent ( Figure 2 ).…”

“…Shunt dimensions were smaller by direct measurement during the operation than by TTE [ 54 ]. Equal results were obtained between cardiac catheterisation and TTE [ 8 ] and between TTE and TEE [ 110 ] in one patient, each. Additionally, identical results of Qp/Qs were obtained by non-invasive (MRI and TTE) and invasive measurements (cardiac catheterisation) in 2 patients [ 25 , 26 ].…”

“…The overall morbidity and mortality were 7.3% (9/123) and 6.5% (8/123), respectively. Eight (9.1%) patients from the acquired group had postoperative complications, including 4 (50% of the complications and 4.5% of the group) atrioventricular block (3 following interventional therapy [ 8 , 43 , 46 ] and 1 following surgical repair [ 104 ]), 2 (25% of the complications and 2.3% of the group) residual shunts and 1 (12.5% of the complications and 1.1% of the group) renal failure; whereas there was only 1 (3.1%) postoperative complication: mild aortic and tricuspid valve insufficiency in the congenital group (χ 2 = 1.2, p = 0.2730). Eight (9.1%) patients died in the acquired group, while no patient died in the congenital group (χ 2 = 3.1, p = 0.0770).…”

“…The retrieval resulted in a collection of 111 articles [ 2 – 113 ], which included 77 (59.3%) case reports [ 3 , 5 – 7 , 9 , 12 – 14 , 16 , 17 , 19 – 23 , 25 – 28 , 30 , 33 , 35 – 37 , 39 , 42 , 44 – 54 , 57 , 59 – 61 , 63 – 67 , 71 , 76 – 80 , 82 , 84 – 89 , 92 – 97 , 99 – 107 , 110 – 112 ] (including 8 (7.4%) case reports in the name of “letter to the editor” [ 5 , 25 , 26 , 30 , 79 , 84 , 92 , 111 ]), 17 (17.6%) medical images [ 2 , 10 , 11 , 15 , 22 , 32 , 40 , 41 , 43 , 58 , 69 , 70 , 72 , 74 , 98 , 108 , 109 ], 5 (4.6%) case series [ 8 , 18 , 34 , 38 ...…”