A case of 75-year old survivor of unrepaired tetralogy of Fallot and quadricuspid aortic valve

Stănescu, Cristina; Branidou, K.

doi:10.1016/j.euje.2007.06.009

Cited by 10 publications

(7 citation statements)

References 17 publications

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“…As the aortic valve normally forms from the embryonic truncus arteriosus, abnormalities in this area may lead to the development of a quadricuspid aortic valve as well as other congenital cardiac defects involving the coronary arteries, the left ventricular outflow tract, the pulmonary valve or the inter-atrial septum [14]. A host of congenital cardiac anomalies has been reported in association with QAV which include, ventricular or atrial septal defect (VSD, ASD) [15,16], pulmonary valve stenosis, sub-aortic fibromuscular stenosis [17], supra-valvular stenosis with left coronary atresia [12], dilatation of the ascending aorta (rarely in contrast to BAV) [8], patent ductus arteriosus [18], tetralogy of Fallot [19,20] with pulmonary atresia [20], hypertrophic obstructive cardiomyopathy [21], aneurysm of Valsalva sinus [22,23] descending aorta-to-pulmonary artery fistula [24] mitral valve prolapse [25], persistent left superior vena cava, right ventricular non-compaction [26] and partial pulmonary venous return anomaly (with ASD) [26].…”

Section: Reported Cardiac and Systemic Associations With Qavsmentioning

confidence: 99%

Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Mehisen¹,

Essely²

2016

J Genet Syndr Gene Ther

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Quadricuspid aortic valve (QAV) is a rare congenital anomaly which is associated with coronary artery anomalies in 10% of the patients. The association of QAV with single coronary artery (SCA) is very rare has been reported only in one case previously. We report the case of a 30-year-old male patient with a unique constellation of congenital anomalies including QAV, SCA, solitary kidney and Tessier type 3 oblique facial cleft with cleft palate. To our knowledge, this unique combination has never been described previously. We describe his case and review the topic of QAV and its reported cardiac and systemic associations.

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Section: Reported Cardiac and Systemic Associations With Qavsmentioning

confidence: 99%

Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Mehisen¹,

Essely²

2016

J Genet Syndr Gene Ther

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“…Here in this case pulmonary orifice diameter is 10 mm so stenosed. [12] According to the finding of Awashthy N, and S. Radhakrishnan in 2013 ventricular size can be determined from Doppler echocardiography. VSD size is mild if it is 1/3 rd of aortic size, moderate if 1/3 rd -2/3 rd of aortic size and large if equal to aortic orifice diameter.…”

Section: Discussionmentioning

confidence: 99%

Adult Tetrology of Fallot: A Doppler Echocardiographic Finding

Deka¹

2021

PLM

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In TOF the morphological abnormalities are -overriding of aorta, pulmonary obstruction, right ventricular hypertrophy and ventricular septal defect. Normally it is nonrestrictive means free communications between the ventricles but sometimes very rarely it can become restrictive when the tricuspid valve restrict the flow across the ventricular septal defect. Among children with CHD 10% of them report TOF. Chest radiographs usually show a normal-size heart silhouette, with an upturned apex and a concave main pulmonary artery segment, commonly known as "boot-shaped" heart. On the electrocardiogram, it is common to see signs of right atrial enlargement and right ventricular hypertrophy showing right axis deviation, prominent R waves anteriorly and S waves posteriorly, upright T wave in V1 (abnormal after 7 days of life up to 10 years of age) and a qR pattern in the right precordial leads. If the ration between pulmonary artery orifice diameter to aortic orifice diameter is <.3 primary repair is unsuccessful and in that case we must go for shunt surgeries which are palliative procedures till permanent repair can be done. This should add proper assessment of coronary artery origin. This is a case of adult tetralogy of fallot (TOF) coming to outpatient department of cardiology with complaints of chest discomfort and sometimes cyanotic spells. Age of the patient is 42 years male. Doppler Echocardiography was done. In the image overriding of aorta was found around 20% over interventricular septum. Left sided aortic arch was detected along with ventricular septal defect (VSD) with size 14 mm. Size of pulmonary orifice 10 mm and that of aortic orifice was 22 mm. Hence pulmonary artery orifice was found to get stenosed.

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“…There are some case reports showing unoperated TOF in elderly (2)(3)(4)(5)(6). Adults develop progressive dyspnea and cyanosis.…”

Section: Discussionmentioning

confidence: 99%

Tetralogy of Fallot May Escape Attention Even in Pregnancy: a Late Presenting Case of a Multiparous Woman

Gül¹,

Abdulhalikov²,

Aslan³

et al. 2014

JAEM

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Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. A 47-year-old woman presented to our clinic with cyanosis and dyspnea on exertion. She had previously had three successful and healthy pregnancies. From the results of laboratory and imaging techniques a diagnosis of tetralogy of Fallot was made and the patient was referred to the cardiovascular surgery for the repairing operation. Here, we report a case of tetralogy of Fallot in a multiparous woman. (JAEM 2014; 13: 36-8)

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A case of 75-year old survivor of unrepaired tetralogy of Fallot and quadricuspid aortic valve

Cited by 10 publications

References 17 publications

Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Adult Tetrology of Fallot: A Doppler Echocardiographic Finding

Tetralogy of Fallot May Escape Attention Even in Pregnancy: a Late Presenting Case of a Multiparous Woman

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