Untreated Tetralogy Of Fallot Research Articles

Right-sided weakness in a Rwandan patient with untreated Tetralogy of Fallot

BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease encountered in pediatrics with surgical repair being the definitive treatment. Long-term survival after surgical repair has improved; however, reported mortality rates in untreated TOF are significant. Associated complications include neurological sequelae such as brain abscess and stroke. In countries without early intervention for congenital heart disease (including TOF), delayed presentations and complications require recognition by healthcare workers.Case presentationA 22-year-old male with a history of untreated TOF presented to Rwanda’s tertiary university hospital, University Teaching Hospital of Kigali, with acute right-sided hemiparesis. Diagnostic imaging identified a left-sided brain lesion consistent with brain abscess and cardiac mass, concerning endocardial vegetation. He was managed with intravenous antibiotics but subsequently died due to complications of septicemia.DiscussionIn countries where surgical repair of TOF is not available, early recognition and medical management are key in temporizing the development of devastating sequelae. Describing the prevalence of CHD in Rwanda is urgent, requiring further research by which effective prevention and treatment strategies can be developed.

Case Report: A forty year-survivor of Tetralogy of Fallot with pulmonary atresia and chronic pediatric shunt thrombosis; findings from cardiac CT scan

Here we illustrate a rare case of Tetralogy of Fallot (TOF) with pulmonary atresia in a 40-year-old survivor patient, despite a chronic pediatric shunt thrombosis. The patient became symptomatic at 38-years-old with progressive dyspnea on exertion and short cyanosis spells. The clinical findings were unspecific except for sinus tachycardia. To reassess cardiac abnormalities and associated intrathoracic malformations, a cardiac CT-scan was performed. Here we explain the mechanism for prolonged survival as an expansion of several and huge major aortopulmonary collateral arteries. This case predicts an exceptional late outcome of untreated TOF.

A 67-year-old men with not corrected tetralogy of Fallot

Tetralogy of Fallot is the most common cyanotic congenital heart disease. Total surgical correction of this defect during infancyallows for long-term survival in most of the patients. The long-term prognosis for untreated tetralogy of Fallot is poor and patients who have not undergone total surgical repair rarely live to old age. We present a case of 67-year-old man with not corrected tetralogy of Fallot diagnosed at the age of 44 years without typical clinical symptoms.

Tet spell in an adult

Cyanosis is a bluish skin due to deoxygenated hemoglobin or methomoglobin in the arterial blood.Central cyanosis mostly results from cardiovascular or pulmonary shunts. Tet spell is an episodic central cyanosis due to total occlusion of right ventricle outflow in a patient with a congenital heart disease, such as Tetralogy of Fallot (TOF). There are limited cases of patients who lived with untreated TOF until adulthood. Tet spell in an adult is rarely seen in an emergency department. Early recognition of this condition, along with proper disposition andmanagement, can prevent majormorbidity or mortality. We report a 29-year-old man who presented with cyanotic spell and in whom a diagnosis of TOF was later confirmed. This case reminds us of TOF as a differential diagnosis of hypercyanotic crises and its unique manifestations and management.

Untreated Tetralogy of Fallot With Pulmonary Atresia

HomeCirculationVol. 113, No. 8Untreated Tetralogy of Fallot With Pulmonary Atresia Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBUntreated Tetralogy of Fallot With Pulmonary AtresiaGiant Aneurysm of an Aortopulmonary Collateral Artery Demonstrated by Magnetic Resonance Imaging Clerio F. Azevedo, MD, Marcelo S. Hadlich, MD, Denilson C. Albuquerque, MD and Jorge N. Moll, MD Clerio F. AzevedoClerio F. Azevedo From the Department of Cardiovascular Magnetic Resonance Imaging, Labs/Rede D’Or Hospitals, Rio de Janeiro, Brazil. Search for more papers by this author , Marcelo S. HadlichMarcelo S. Hadlich From the Department of Cardiovascular Magnetic Resonance Imaging, Labs/Rede D’Or Hospitals, Rio de Janeiro, Brazil. Search for more papers by this author , Denilson C. AlbuquerqueDenilson C. Albuquerque From the Department of Cardiovascular Magnetic Resonance Imaging, Labs/Rede D’Or Hospitals, Rio de Janeiro, Brazil. Search for more papers by this author and Jorge N. MollJorge N. Moll From the Department of Cardiovascular Magnetic Resonance Imaging, Labs/Rede D’Or Hospitals, Rio de Janeiro, Brazil. Search for more papers by this author Originally published28 Feb 2006https://doi.org/10.1161/CIRCULATIONAHA.105.550582Circulation. 2006;113:e293–e294A 41-year-old man with untreated tetralogy of Fallot and pulmonary atresia was referred to our department for imaging. His diagnosis had been established in early infancy, but cardiac surgery was repeatedly refused over the years. In addition to central cyanosis and clubbing of the fingers, the patient started experiencing worsening exertional dyspnea, cough, and pain in the back and left shoulder within the last 2 years. A transthoracic echocardiogram revealed, in addition to the findings consistent with his congenital heart disease, a large paracardiac mass in the left hemithorax. To evaluate the cardiac morphology in greater detail and to better characterize the paracardiac mass, magnetic resonance imaging was performed (Figure 1 through Figure 3 and Data SupplementMovie I through Movie III). The typical features of tetralogy of Fallot, such as outlet ventricular septal defect, overriding aorta, right ventricular (RV) hypertrophy, and obstruction to the RV outflow, were readily identified. In addition, this patient displayed extensive aortopulmonary collateral circulation, with a large aneurysmatic dilatation of the left internal mammary artery (a major aortopulmonary collateral vessel in this case). Download figureDownload PowerPointFigure 1. Steady-state, free-precession cine magnetic resonance images depicting cardiac morphology. A, Four-chamber view demonstrates the ventricular septal defect with overriding aorta (arrowhead), RV hypertrophy (straight arrow), and moderate pericardial effusion (curved arrow). B, Coronal view showing the characteristic boot-shaped heart (coeur en sabot) of tetralogy of Fallot and the giant aneurysm of the aortopulmonary collateral artery positioned in the left hemithorax above the heart (*).Download figureDownload PowerPointFigure 2. Coronally targeted, maximum-intensity projection images of the contrast-enhanced 3-dimensional magnetic resonance angiograms. Note the significant dilatation of the proximal portion of the left subclavian artery (arrowhead) and left internal mammary artery (LIMA), which gives rise to the aneurysm (straight arrow). The left subclavian artery demonstrates a normal diameter after the origin of the LIMA (curved arrow). The site of aneurysmatic dilatation (*) is shown just before (A) and immediately after (B) contrast agent arrival at the aneurysm.Download figureDownload PowerPointFigure 3. Coronally targeted, volume-rendered images of the contrast-enhanced 3D magnetic resonance angiograms. A, Anterior view; B, posterior view. Note the absence of pulmonary arteries (pulmonary artery atresia) and the extensive aortopulmonary collateral circulation (arrow). From the posterior wall of the aneurysm, the distal portion of the left internal mammary artery progresses to connect with the pulmonary circulation (arrowhead).The online-only Data Supplement can be found at http://circ.ahajournals.org/cgi/content/full/113/8/e293/DC1.DisclosuresNone.FootnotesCorrespondence to Clerio F. Azevedo, MD, Department of Cardiovascular Magnetic Resonance Imaging, Labs/Rede D’Or Hospitals, Rua Homem de Melo 126/201, Rio de Janeiro, RJ, Brazil. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By (2009) Tetralogy of Fallot Congenital Heart Defects, 10.1007/978-3-7985-1719-6_8, (56-67), . February 28, 2006Vol 113, Issue 8 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.105.550582PMID: 16505180 Originally publishedFebruary 28, 2006 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseImaging

手術治療なしで長期延命したFallot四徴症の女性の一例

It has been reported that most patients with untreated tetralogy of Fallot (TOF) die by the time they reach adulthood. We report the case of a 72-year-old female diagnosed by echocardiography and cardiac cathetherization as having TOF and diagnosed at birth with a ventricular septal defect (VSD). During childhood, she was very thin and lacking in physical strength. On first consultation at our hospital, she was suffering from mild dyspnea, classified as NYHA functional class III, and her fingers were clubbed and cyanotic. Her PaO2 was 48.0 mmHg under room air, and hypoxia was recognized. An echocardiography and cardiac cathetherization showed a VSD, hypertrophy of the right ventricle, over-riding of the aorta and stenosis of the right ventricular outflow tract with a pressure gradient of 84 mmHg. There was a bidirectional shunt with 24% flow from the left to right and 43% from the right to left ventricle. Her Qp/Qs was 0.75. Surgical treatment was recommended. However, the patient refused, because her symptoms were alleviated with home oxygen therapy. This report shows the prolonged survival of this 72-year-old female with untreated TOF.

Untreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old Woman

HomeCirculationVol. 110, No. 18Untreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old Woman Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBUntreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old WomanFindings From Magnetic Resonance Imaging Michael Puderbach, Joachim Eichhorn, Christian Fink and Hans-Ulrich Kauczor Michael PuderbachMichael Puderbach From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author , Joachim EichhornJoachim Eichhorn From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author , Christian FinkChristian Fink From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author and Hans-Ulrich KauczorHans-Ulrich Kauczor From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author Originally published2 Nov 2004https://doi.org/10.1161/01.CIR.0000146789.79192.08Circulation. 2004;110:e461–e462A 55-year-old woman with congenital heart disease and increasing dyspnea at low levels of exertion was referred to our department for imaging. Clinical findings were cyanosis of the lips and clubbing of the fingers. The past medical history included several hospitalizations for dyspnea, tachycardia, recurrent pulmonary embolism, and suspected cerebral embolism.Despite several conventional angiographic studies, the exact classification of the congenital heart disease and the vascular abnormalities had not been made because of the complex cardiovascular morphology. The presumptive diag- nosis was truncus arteriosus type IV. Differential diagnosis included tetralogy of Fallot with pulmonary atresia.To reevaluate the morphology and to assess acute pulmonary, cardiac, and thoracic vessel problems, MRI was performed (Figures 1 to 4). It revealed a ventricular septal defect with overriding aorta, pulmonal atresia with collateral blood supply by major aortopulmonary collateral arteries, and collateral arteries originating from the right phrenic arteries and intercostal arteries. These findings are consistent with tetralogy of Fallot with pulmonary atresia. Download figureDownload PowerPointFigure 1. Coronal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Lack of pulmonary arteries in pulmonary atresia. Visualization of collateral blood supply of the lungs: Major aortopulmonary collateral arteries (MAPCA) (black arrow) and additional collateral arteries originating from the right phrenic arteries (white arrow) and intercostal arteries (curved arrow).Download figureDownload PowerPointFigure 2. Coronal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Visualization of predominantly right-sided dilated intercostal arteries representing collateral blood supply to the right lung in pulmonary atresia.Download figureDownload PowerPointFigure 3. Parasagittal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Visualization of the ventricular septal defect (arrow) with overriding aorta in tetralogy of Fallot.Download figureDownload PowerPointFigure 4. Selected images of 4-chamber view from cardiac cine MRI over the cardiac cycle. Visualization of the ventricular septal defect with overriding aorta. Jet over the ventricular septal defect from the right to the left ventricle representing Eisenmenger’s reaction. LA indicates left atrium; RA, right atrium; LV, left ventricle; RV, right ventricle; Ao, aorta; arrow, ventricular septal defect; and open arrow, jet over the ventricular septal defect).The online-only Data Supplement, which contains Movies I and II, is available with this article at http://www.circulationaha.org.FootnotesCorrespondence to Michael Puderbach, MD, Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg, Germany. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By (2009) Tetralogy of Fallot Congenital Heart Defects, 10.1007/978-3-7985-1719-6_8, (56-67), . November 2, 2004Vol 110, Issue 18 Advertisement Article InformationMetrics https://doi.org/10.1161/01.CIR.0000146789.79192.08PMID: 15520329 Originally publishedNovember 2, 2004 PDF download Advertisement SubjectsAngiographyComputerized Tomography (CT)Heart Failure

Untreated tetralogy of Fallot in an adult patient complicated by acute aortic valve endocarditis

Acute endocarditis complicated by aortic valve regurgitation is an uncommon finding in adults with surgical untreated tetralogy of Fallot (TOF). The hemodynamic consequences for both, the right and left ventricles, are significant. However, the right ventricle may be in a disastrous situation, since a compromised right ventricle from longstanding pressure overload may not tolerate acute volume overload. Here we report a 28-year-old African adult patient with TOF and acute severe aortic valve regurgitation due to bacterial endocarditis with preoperative low cardiac output syndrome. After aortotomy a large abscess cavity underneath the left and non-coronary sinus and a TOF typical perimembranous ventricular septal defect (VSD) were visible. Autologous glutaraldehyde-treated pericardium was used to reconstruct the aortic-mitral curtain. For repair of the TOF a vertical incision in the right ventricular outflow tract was performed and the infundibular septum was resected. The perimembranous VSD was closed with glutaraldehyde-fixed autologous pericardium, whereas the cranial part of the patch formed the aortic annulus. An aortic homograft was implanted as a full aortic root. The patient recovered early and uneventful. Adult TOF may be complicated by acute aortic valve endocarditis with emergent surgical intervention. Homograft aortic valve replacement is feasible in this setting.