Persistent valve of systemic venous sinus: a cause of neonatal cyanosis

Qureshi, Ahmad Usaid; Latiff, Haifa Abdul; Sivalingam, Sivakumar

doi:10.1017/s1047951113001200

Cited by 6 publications

(13 citation statements)

References 9 publications

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“…1 Persistent valve of systemic venous sinus, in its simplest form as a prominent or giant EV, is a common finding. 2 The Chiari network represents a more incomplete involution of the embryonic structure. 2 Cor triatriatum dexter is the most severe form with no or minimal involution of valve tissue.…”

Section: Discussionmentioning

confidence: 99%

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Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager

Kernizan

Kharouf

Robinson

et al. 2020

World J Pediatr Congenit Heart Surg

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Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

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Section: Discussionmentioning

confidence: 99%

“…2 The Chiari network represents a more incomplete involution of the embryonic structure. 2 Cor triatriatum dexter is the most severe form with no or minimal involution of valve tissue. 2…”

Section: Discussionmentioning

confidence: 99%

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager

Kernizan

Kharouf

Robinson

et al. 2020

World J Pediatr Congenit Heart Surg

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“…Remnants of the right valve of the sinus venosus in infants have been managed conservatively with spontaneous resolution of symptoms [7], or with surgery, in cases where hypoxia was persistent [8,9]. No cases to date have explored management decisions in a neonate with a functional cor triatriatum dexter who suffered embolic stroke.…”

Section: Discussionmentioning

confidence: 99%

Cyanosis and Stroke due to Functional Cor Triatriatum Dexter in a Neonate

et al. 2018

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Small remnants of the right valve of the sinus venosus are commonly found in adults, but the incidence and risk associated with these embryonic remnants in neonates are not well studied. The following report describes a cyanotic neonate with a large Eustachian valve remnant creating a functional cor triatriatum dexter who was initially diagnosed with persistent pulmonary hypertension of the newborn. The cyanosis in this infant improved over the first postnatal week with conservative management, but she suffered multifocal subcortical stroke, likely related to her intracardiac shunt. The clinical presentation and questions regarding long-term management of this rare diagnosis are explored.

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“…On this basis, it proved possible to make an individualized review of 193 investigations 1–193 . These findings were then incorporated, as far as possible, with the results of a systematic review of ...…”

Section: Methodsmentioning

confidence: 99%

“…Division of the morphologically left atrium has been estimated to be around 0.1% to 0.4%, while the persistence of the valves of the systemic venous sinus accounts for 0.025%–0.1% of congenital heart diseases 22,29–31 . Associated congenital and acquired cardiac lesions have been reported in one‐third to three‐quarters of those with divided left atriums and in one‐half of those in whom the morphologically right atrium is divided 23–28,31–91 . Limited information is available, however, on the short‐ and long‐term outcomes of treatment for these rare conditions.…”

Section: Introductionmentioning

confidence: 99%

Surgical management of divided atrial chambers

Sankhyan

Anderson

Chowdhury

et al. 2021

Journal of Cardiac Surgery

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Background and Aim The morphological variations when one, or both, of the atrial chambers is subdivided, are many and varied. We sought to address clinical presentations, potentially misdiagnosed cases, diagnostic modalities, surgical approaches, and outcomes of this “family” of uncommon lesions. Methods A total of 193 published investigations were synthesized. Diagnostic information was provided by clinical presentation, and multimodality imaging studies. Results Almost three‐quarters of patients with divided atrial chambers present during infancy with severe pulmonary hypertension and cardiac failure. Associated cardiac and extra‐cardiac defects are present in between half and nine‐tenths of cases. Acquired division of the left atrium has been reported after the Fontan operation, orthotopic cardiac transplantation, and complicated aortic valvar infective endocarditis. Surgery under cardiopulmonary bypass remains the definitive treatment. Balloon dilation may be considered in anatomically compatible variants in the setting of cardiac failure and pregnancy as a bridge todefinitive treatment. Overall, mortality has been cited between nil to 29%. Presentation during infancy, associated congenital anomalies, pulmonary hypertension, and surgery in the previous era, have been the reported causes of death. The operative survivors have long‐term favourable outcomes, with near normal cardiac dimensions and low risk of recurrence. While asymptomatic patients with division of the right atrium do not need treatment, surgical resection of the dividing partition under cardiopulmonary bypass is recommended in symptomatic patients with complex anatomy, the spinnaker malformation, or associated cardiac anomalies. Balloon dilation may be considered in uncomplicated patients with less obstructive lesions. Hybrid intervention and endoscopic robotic correction also have been performed. Conclusions Resection of the dividing shelf allows the survivors to regain near normal dimensions with a low risk of recurrence. We submit that an increased appreciation of the anatomic background to division of the atrial chambers will contribute to improved surgical management.

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Persistent valve of systemic venous sinus: a cause of neonatal cyanosis

Cited by 6 publications

References 9 publications

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager

Cyanosis and Stroke due to Functional Cor Triatriatum Dexter in a Neonate

Surgical management of divided atrial chambers

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