Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks

Bharati, Alpa; Naware, Ajita; Merchant, S

doi:10.4103/0971-3026.43841

Cited by 9 publications

(7 citation statements)

References 4 publications

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“…Unlike others, TOF patients with an absent pulmonary valve show aneurysmal dilatation of the pulmonary trunk and branch pulmonary arteries,[10] with mild stenosis at the level of the pulmonary annulus. This was seen in two of the patients in this study.…”

Section: Discussionmentioning

confidence: 89%

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Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot

Chacko

Chiramel

Vimala

et al. 2017

Indian Journal of Radiology and Imaging

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Background:Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail.Aims:The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk.Materials and Methods:The cardiac CT scans of 30 patients with TOF were reviewed to evaluate the morphology of the pulmonary valve, infundibulum, and pulmonary arteries. Fisher's exact test was performed to examine the association between pulmonary valve morphology and degree of hypoplasia of the infundibulum and pulmonary trunk.Results:16.7% of patients with TOF had pulmonary atresia. The prevalence of tricuspid, bicuspid, and absent valves were 10%, 53.3% and 6.7%, respectively. In another 13.3% of patients, although valve tissue was present, exact morphology could not be determined on CT. The commissures of 62.5% of the bicuspid valves were at 12 o’clock and 6 o’clock or slightly off the midline. There was statistically significant association between valve morphology and degree of infundibular hypoplasia (P < 0.001) and calibre of pulmonary trunk (P < 0.001).Conclusion:Morphological abnormality of the pulmonary valve is common in TOF. The most common type of pulmonary valve in TOF patients is bicuspid valve with commissures at 12 o’clock and 6 o’clock or slightly off the midline. Fewer cusps of the pulmonary valve are associated with a more severe degree of pulmonary artery hypoplasia.

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Section: Discussionmentioning

confidence: 89%

“…Both these patients had normal-sized infundibulum. The aneurysmal pulmonary artery is known to cause tracheobronchial compression[10] and severe obstructive airway disease in these cases.…”

Section: Discussionmentioning

confidence: 99%

Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot

Chacko

Chiramel

Vimala

et al. 2017

Indian Journal of Radiology and Imaging

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“…Nearly one-fourth of the cases TOF/APVS are associated with other CHD such as dextrocardia,2 atrioventricular septal defects,3 and others. We report a novel association of TOF/APVS with an abnormal origin of the LPA from the ascending aorta (hemitruncus).…”

Section: Discussionmentioning

confidence: 99%

“…Although TOF is the most commonly associated congenital heart defect (CHD) in APVS, other defects have been described 2,3. Absence of a patent ductus arteriosus (PDA) has been postulated as a cause of APVS 4.…”

mentioning

confidence: 99%

Absent Pulmonary Valve Syndrome with Left Hemitruncus

et al. 2012

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Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta.

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“…In this paper, we wanted to remember other cardiac anomalies associated with absent pulmonary valve syndrome with tetralogy of Fallot. Çeşitli çalışmalar yapılmış ve PKYS'ye eşlik edebilecek anomaliler yayınlanmıştır: Sol pulmoner arter yokluğu, sol pulmoner arterin PDA'dan çıkması, sağ pulmoner arterin asendan aortadan çıkması ve sağ arkus aorta, triküspit atrezisi, sol pulmoner arterin asendan aortadan çıkması, sol ana koroner arter atrezisi, aort koarktasyonu, Ebstein anomalisi, dekstrokardi, atriyoventriküler septal defekt, büyük arterlerin transpozisyonu, trikuspit atrezisi veya ağır triküspit stenozu, intakt ventrikular septum, kesintili aortik ark tip B, aortopulmoner kollateraller, double aortik ark ile birliktelik bildirilmiştir (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Section: Olguunclassified

A Rare Cardiac Anomaly in Absent Pulmonary Valve Syndrome with Tetralogy of Fallot

Akgün¹,

Ergin²,

Yılmaz³

et al. 2018

Haseki

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Pulmoner kapak yokluğu sendromu (PKYS), Fallot tetralojisine (FT) benzer fakat farklı bir klinik tablodur. Anatomik özellik olarak rudimente pulmoner kapak (hem darlık hem yetersizlik olan), masif dilate pulmoner arterler ve sıklıkla geniş ventriküler septal defekti (VSD) içerir. Hemen daima patent duktus arteriosus (PDA) yokluğu ile beraberdir. Çocukluk formu iyi seyretmesine rağmen infant formu ciddi solunum problemleri ile beraber olup erken cerrahi (ilk üç-altı ay) gerektirir (1). Ya z›fl maAd re si/Ad dressforCor res pon den ce:Taliha Öner

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Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks

Cited by 9 publications

References 4 publications

Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot

Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot

Absent Pulmonary Valve Syndrome with Left Hemitruncus

A Rare Cardiac Anomaly in Absent Pulmonary Valve Syndrome with Tetralogy of Fallot

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