The double outlet right ventricle

Objective. Aortic arch obstruction is a commonly associated problem in the Taussig-Bing anomaly. Between 1983 and 1995, 28 consecutive patients with Taussig-Bing anomaly underwent arterial switch operation with baffling of the left ventricle to neoaorta. Group A: 11/28 had associated aortic arch obstruction. Group B: 17/28 had isolated Taussig-Bing anomaly. We assessed whether the coexistence of subpulmonary ventricular septal defect and aortic arch obstruction pose an incremental risk factor. Patients and results. Group A: Mean age and weight were 1.4±1.3 months and 3.5±0.4 kg. The aortic arch obstruction included: hypoplasia (5/11), interruption (4/11) and discrete coarctation (2/11). Seven patients had a one-stage correction, and four had initial arch repair followed by arterial switch operation. There were no hospital deaths (CL 0-28%). Over a follow-up of 638 patient-months (mean 64±39), there have been no late deaths, and all patients are in New York Heart Association class 1. There have been three cases of recurrent aortic arch obstruction (two requiring reoperation, and one requiring balloon dilation). One patient has been reoperated upon for right ventricular outflow tract obstruction. The actuarial survival and freedom from reoperation rates at 6 years were 100% (CL=66-100%) and 72.9% (CL=38-92%) respectively. Group B: Mean age and weight were 5.9 ± 8.4 months and 5±2.1 kg. All patients had a one-stage operation. There were two early deaths (11.8%, CL=1-36%) and one late death over a follow-up of 678 patient-months (mean 52±31). All survivors are in New York Heart Association class 1 and there have been no reoperations. The actuarial survival and freedom from reoperation rates at 6 years were 81% (CL=56-93%) and 100% (CL=76-100%) respectively. Conclusions. 1. Aortic arch obstruction has not adversely affected early or late survival (P>.05) or late functional class. 2. Patients with Taussig-Bing anomaly and aortic arch obstruction may have a higher reoperation rate than those with normal arch anatomy. 3. Taussing-Bing anomaly, with or without aortic arch obstruction, can be repaired with arterial switch operation during the neonatal period with good outcome.

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Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome.

Coarctation of the aorta (CoA) is defined as a congenital stenosis of the thoracic aorta and is one of the most common congenital cardiovascular diseases. Despite successful surgical treatment for CoA, arterial abnormalities, including refractory hypertension, aortic aneurysm, and proatherogenic phenotypic changes, frequently affect patients’ quality of life. Emerging evidence from morphological and molecular biological investigations suggest that the area of CoA is characterized by phenotypic modulation of smooth muscle cells, intimal thickening, and impaired elastic fiber formation. These changes extend to the pre-and post-stenotic aorta and impair arterial elasticity. The aim of this review is to present current findings on the pathology and molecular mechanisms of vascular remodeling due to CoA. In particular, we will discuss the association between CoA and the ductus arteriosus since the most common site for the stenosis is in the proximity of the ductus arteriosus.

/pdf/pathology-and-molecular-mechanisms-of-coarctation-of-the-4eag5xvczu.pdf

Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus

A new operation for d-loop transposition of the great vessels

Coarctation of the aorta is a congenital heart disease, which is often associated with other cardiac and noncardiac anomalies. Early diagnoses, information about associated anomalies, and defining the severity of the disease are critical for appropriate treatment planning. In this regard, several noninvasive imaging modalities, such as echocardiography, cardiac computed tomography (CT), and cardiac magnetic resonance imaging, have been used. Echocardiography, as an available and safe method, should be used as a primary screening test. It is also useful for intraoperative and hemodynamic studies, but cardiac CT is recommended before any corrective procedure or surgery. Cardiac CT angiography showed an excellent spatial resolution and a good capability for finding associated anomalies. After correction of coarctation of the aorta, serial cardiac magnetic resonance imaging is most commonly performed to avoid repeated radiation exposure.

Use of noninvasive imaging in the evaluation of coarctation of aorta.

The coexistence of the Taussig-Bing anomaly and coarctation of the aorta is a highly complex situation carrying a dismal prognosis. Through our experience and a review, we have observed that neonates requiring coarctation repair, pulmonary artery banding, and patent ductus ligation are at high risk of expiring before reaching an age at which a difficult total repair is feasible. It appears that patients presenting beyond the neonatal period have a better chance of surviving an initial surgical procedure and the definitive repair. A surgical management protocol has been suggested. Although associated with an uncertain late prognosis, arterial level repairs are the most physiologic, and their results to date are encouraging.

Taussig-Bing anomaly and coarctation of the aorta in infancy: surgical options.

LBA53 ELIOS: A multicentre, molecular profiling study of patients (pts) with epidermal growth factor receptor-mutated (EGFRm) advanced NSCLC treated with first-line (1L) osimertinib

Recent data suggest that the overall survival (OS) and progression-free survival (PFS) observed in RW patients with mNSCLC receiving IO regimens may be shorter than that seen in randomised clinical trials (RCTs). This retrospective, observational study describes rwOS and rwPFS (overall and by PD-L1 expression) compared with the outcomes observed in RCTs in patients with mNSCLC. 

107P Outcomes of patients with metastatic non-small cell lung cancer (mNSCLC) receiving first-line (1L) immunotherapy (IO) with or without chemotherapy (CT): Real-world (RW) evidence vs clinical trial results - CORRELATE

170P Preliminary results from LUMINANCE: A phase IIIb, single-arm study of 1L durvalumab (D) + platinum-etoposide (EP) for patients with extensive-stage SCLC (ES-SCLC)

LBA12 Tumour genomics in patients (pts) with epidermal growth factor receptor-mutated (EGFRm) advanced NSCLC treated with first-line (1L) osimertinib in the phase II ELIOS study

Samuel H. Sadow

Papers

Taussig-Bing anomaly and coarctation of the aorta in infancy: surgical options.

LBA53 ELIOS: A multicentre, molecular profiling study of patients (pts) with epidermal growth factor receptor-mutated (EGFRm) advanced NSCLC treated with first-line (1L) osimertinib

107P Outcomes of patients with metastatic non-small cell lung cancer (mNSCLC) receiving first-line (1L) immunotherapy (IO) with or without chemotherapy (CT): Real-world (RW) evidence vs clinical trial results - CORRELATE

170P Preliminary results from LUMINANCE: A phase IIIb, single-arm study of 1L durvalumab (D) + platinum-etoposide (EP) for patients with extensive-stage SCLC (ES-SCLC)

LBA12 Tumour genomics in patients (pts) with epidermal growth factor receptor-mutated (EGFRm) advanced NSCLC treated with first-line (1L) osimertinib in the phase II ELIOS study