Partial anomalous pulmonary venous connection (including scimitar syndrome)

Woestijne, Pieter C. van de; Verberkmoes, Niels; Bogers, Ad J.J.C.

doi:10.1093/mmcts/mmt001

Cited by 7 publications

(12 citation statements)

References 3 publications

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“…The chief pathophysiological mechanism includes the development of left-to-right shunt due to the abnormal return of one or more right pulmonary vein(s), which depends on the pulmonary lobe or segments from which the anomalous vein originates, number of pulmonary veins involved, and relative resistance of draining pulmonary beds [6]. This shunt eventually induces volume overload on the right-sided heart, thus causing heart failure [7].…”

Section: Discussionmentioning

confidence: 99%

Scimitar syndrome – Uncommon variant with common presentation

Dave¹,

Parikh²,

Vasoya³

2020

IJCR

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S cimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature of partial or total anomalous pulmonary venous drainage of the right lung to the systemic circulation [1]. It is characterized by partial anomalous pulmonary venous drainage of the right lung causing left-to-right shunt along with the right lung hypoplasia, dextraposition of the heart [2], and pulmonary hypertension due to aortopulmonary collaterals [3]. When visualized on imaging, it resembles a backsword with a curved blade, also known as scimitar in Middle East [4]. In infants, it can present as recurrent lung infection and can develop complications such as pulmonary arterial hypertension and congestive cardiac failure [5], while in adults, it usually remains asymptomatic [6]. It can be associated with other congenital heart defects such as single ventricular defect causing congestive cardiac failure [7] or atrial septal defects [8]. The surgical treatment includes rightsided pneumonectomy [9] or, a tunnel connecting the scimitar vein, and the left atrium can be made by Dacron graft through intra-atrial baffle technique [10]. CASE REPORT A 9-month-old male baby presented to the pediatric outpatient clinic with complaints of fever with cough, cold, and decreased oral intake for 5 days. His vitals demonstrated low-grade fever of 98.2°F with a pulse rate of 110/min, and respiratory rate of 36/ min. On physical examination, he looked pale and crepitations were heard over his chest. Therefore, he was admitted and commenced on IV antibiotics and nebulization. The laboratory findings showed anemia (6.70 g/dl), leukocytosis with counts of 13,200/mm 3 , and elevated C-reactive protein (CRP) levels of 23.40 mg/dl. The chest X-ray showed diffuse changes of bronchitis. His further laboratory investigations are mentioned in Table 1. His clinical condition improved over a period of time and there was a decrease in the count and CRP levels. His oral intake also improved gradually and thus was discharged after 3 days with the prescription of oral antibiotics. Following this, he had four bouts of respiratory tract infections with similar complaints and was treated on an outpatient basis. He underwent a 32 slice computed tomography (CT) scan with IV contrast which demonstrated non-visualization of the right main pulmonary artery and smaller right lung. The 2D echocardiography confirmed the hypoplastic right pulmonary artery with a small left-to-right patent ductus arteriosus shunt. The aortopulmonary collaterals were noted supplying to the right lung in the absence of the right pulmonary artery. These findings were in accordance with the scimitar syndrome variant with preferential blood flow to the left lung causing mild pulmonary artery hypertension.

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Section: Discussionmentioning

confidence: 99%

Scimitar syndrome – Uncommon variant with common presentation

Dave¹,

Parikh²,

Vasoya³

2020

IJCR

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“…The reason behind the higher risk of iatrogenic SA node injury using the double-patch repair technique lies intuitively in the need for a more extensive superior cavo-atrial incision, which is a peculiar feature of this operation (7). In this surgical scenario, the possibility of damaging the SA node is certainly increased than in other procedures where the surgeon remains far away from this area, such as in the Warden and in the double-decker operations (10;15).…”

Section: Discussionmentioning

confidence: 99%

“…The "double-patch" technique is generally chosen instead when the PVs are connected to the SVC above the cavo-atrial junction. In these instances, after the ASD closure and the PVs rerouting, an additional pericardial patch is used to enlarge the cavo-atrial junction, thus preventing SVC stenosis (7). The Warden procedure, originally described in 1984 (8), is a useful technique that can be performed when the PVs connection lies above the superior cavo-atrial junction.…”

Section: Introductionmentioning

confidence: 99%

The impact of the superior cavo-atrial incision in children and adults.

Ait-Alì¹,

Ravaglioli²,

Festa³

et al. 2020

Preprint

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Background and aim of the study The single-and double-patch repairs are the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in pediatric and adult patients, focusing in particular on the occurrence of postoperative rhythm disturbances. Methods 115 patients (male: 63, 54.8%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. 80 patients (70.4%) underwent surgical repair in pediatric age (<16 years old), while 35 (29.6%) patients were operated in adulthood. 49 patients (42.6%) underwent single-patch repair and 65 patients (56.5%) underwent double-patch repair. In only one patient the Warden procedure was performed (0,9%). Median follow-up time was 52 months (IQ 15.1-113). Results The type of surgical technique didn't affect the overall incidence of all rhythm disturbances (41% in single patch group and 35% in double patch groups, p=0.5). At long-term follow-up, ectopic atrial rhythm, as expression of sinoatrial node disturbance, was however significantly more frequent in the pediatric population (32% pediatric group and 8% adult group, p=0.02). Conclusions The higher incidence of ectopic atrial rhythm in children is probably related to the closer relationship of the sinus node to the superior cavo-atrial incision, which makes iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavo-atrial junction should therefore be preferred for children undergoing partial anomalous pulmonary venous connection repair. The impact of the superior cavo-atrial incision in children and adults.

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“…3, 4 and 5) [12]. The identification of these abnormalities has a significant bearing on surgical management as anomalous pulmonary veins could be rerouted to the left atrium via an atrial septal defect [13].…”

Section: A Partial Anomalous Pulmonary Venous Drainage (Papvd)mentioning

confidence: 99%

Anomalous pulmonary venous drainage: a pictorial essay with a CT focus

Lyen

Wijesuriya

Ngan-Soo

et al. 2017

J Congenit Heart Dis

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The imaging of pulmonary venous anatomy has traditionally been performed with echocardiography and catheter pulmonary angiography. Magnetic resonance imaging (MRI) and notably multi-detector row computed tomography (CT) have refined the usual imaging techniques. Anomalous pulmonary venous drainage (APVD) is the drainage of one or more pulmonary veins outside the left atrium. Its detection is critical due to the strong association with congenital heart disease as well as other cardiac and respiratory anomalies, which have significant implications for patient management. The pervasive application of CT combined with the relatively non-specific clinical presentation of APVD has resulted in the increased incidental detection of these anomalies. Knowledge is hence vital as the imaging specialist is now usually the first person to make such a diagnosis. Furthermore, pulmonary veins are an important site for arrhythmogenic foci and radiofrequency ablation of such sites is used in the treatment of refractory atrial fibrillation. Hence an imaging road map of these veins is crucial before any management can take place. This pictorial review will illustrate embryology, normal and variant pulmonary vein anatomy, and varied patterns of APVD. Finally, we discuss the implications in the treatment of atrial fibrillation.

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Partial anomalous pulmonary venous connection (including scimitar syndrome)

Cited by 7 publications

References 3 publications

Scimitar syndrome – Uncommon variant with common presentation

Scimitar syndrome – Uncommon variant with common presentation

The impact of the superior cavo-atrial incision in children and adults.

Anomalous pulmonary venous drainage: a pictorial essay with a CT focus

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