Absent pulmonary valve syndrome with tetralogy of Fallot detected at an early gestational age of 27 weeks – A case report

Bhupali, Ashok; Patankar, Kiran B.; Prasad, Sayi; Patil, Jeetendra K.; Tamhane, Ajitey

doi:10.1016/j.ihj.2012.10.010

Cited by 12 publications

(7 citation statements)

References 5 publications

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“…TOF is not only an anatomic anomaly, but it also seems to be a complicated genetic disorder that can be associated with other anomalies such as an absent pulmonary valve, the absence of a pulmonary artery, and extracardiac anomalies [22,23]. Most patients with APA have pulmonary artery hypertension [5,10].…”

Section: Discussionmentioning

confidence: 99%

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

Edraki

Ghasemzadeh

Keshavarz

et al. 2021

BMC Cardiovasc Disord

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Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

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Section: Discussionmentioning

confidence: 99%

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

Edraki

Ghasemzadeh

Keshavarz

et al. 2021

BMC Cardiovasc Disord

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show abstract

Section: Discussionmentioning

confidence: 99%

Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery

Edraki

Ghasemzadeh

Keshavarz

et al. 2020

Preprint

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Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

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“…APVS is associated with airway compression which continues to remain a challenge. Compression against bronchial tree and esophagus due to dilated pulmonary artery and its branches may inevitably lead to bronchomalacia and polyhydramnios [ 3 ]. In their series including 21 fetal cases with absent pulmonary artery, Volpe et al detected 22q11 microdeletion in 25% of the cases [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia

Seven

Yalınbaş

Özdemir

2016

Case Reports in Obstetrics and Gynecology

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Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examination of the fetus at the 26th week of gestation revealed severe polyhydramnios, dilatation at right ventricle, and abnormal appearance of the heart. At the 31st gestational week, the baby was born with cesarean section. The newborn had right heart failure but had no hydrops fetalis. Therefore, severe respiratory distress observed in the infant has been associated with pulmonary complications. The infant, who had respiratory acidosis according to blood gas analysis, was intubated and attached to mechanical ventilator. Despite progressively increased respiratory support and other interventions, the infant died on the 3rd day of admission. Compression against bronchial tree and esophagus due to dilated pulmonary artery and its branches may inevitably lead to bronchomalacia and polyhydramnios. In conclusion, presence of polyhydramnios and the possibility of severe bronchomalacia should be kept in mind; and due to the risk of early neonatal mortality, delivery should be performed in a center where pediatric heart surgery is available.

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Absent pulmonary valve syndrome with tetralogy of Fallot detected at an early gestational age of 27 weeks – A case report

Cited by 12 publications

References 5 publications

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery

A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia

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