scholarly journals Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis

Author(s):  
Anjali Chelliah ◽  
Anita J. Moon‐Grady ◽  
Shabnam Peyvandi ◽  
Joanne S. Chiu ◽  
James E. Bost ◽  
...  

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P =0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P =0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

2017 ◽  
Vol 28 (2) ◽  
pp. 208-213 ◽  
Author(s):  
Pekka Ylitalo ◽  
Eero Jokinen ◽  
Kirsi Lauerma ◽  
Miia Holmström ◽  
Olli M. Pitkänen-Argillander

AbstractBackgroundRight ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus.MethodsThe study group comprised 38 patients with tetralogy of Fallot who underwent surgical repair between 1990 and 2003. Transannular patching was required in 21 patients to reconstruct the right ventricular outflow tract. Altogether, 48 age- and gender-matched healthy volunteers were recruited. Cardiac MRI was performed on all study patients to assess the atrial and ventricular volumes and function.ResultsSevere pulmonary regurgitation (>30 ml/m2) was present in 13 patients, of whom 11 had a transannular patch, but only two had a preserved pulmonary valve annulus. The ventricular preload volumes from both atria were significantly reduced in patients with severe pulmonary regurgitation, and left ventricular stroke volumes (44.1±4.7 versus 58.9±10.7 ml/m2; p<0.0001) were smaller compared with that in patients with pulmonary regurgitation <30 ml/m2or in controls.ConclusionsIn patients with tetralogy of Fallot, severe pulmonary regurgitation has a significant effect on volume flow through the left atrium. Reduction in left ventricular preload volume may be an additional factor contributing to left ventricular dysfunction.


2002 ◽  
Vol 73 (6) ◽  
pp. 1794-1800 ◽  
Author(s):  
Frank T.H. de Ruijter ◽  
Ineke Weenink ◽  
Francois J. Hitchcock ◽  
Erik J. Meijboom ◽  
Ger B.W.E. Bennink

2021 ◽  
Vol 33 (1) ◽  
Author(s):  
Ahmed Aly Obiedallah ◽  
Ashraf Anwar E. L. Shazly ◽  
Noura Gamal Nasr ◽  
Essam M. Abdel Aziz

Abstract Background Heart failure (HF) is a major health problem. Cardiac and renal diseases interact in a complex bidirectional manner in both acute and chronic settings. Renal dysfunction in the setting of heart failure, termed the cardio renal syndrome (CRS), has been considered consequence of left ventricular dysfunction (LVD), whereby decreasing cardiac output (COP) results in renal under perfusion and consequent decreased glomerular filtration rate (GFR). Main body of the abstract This study showed that 500 patients were admitted to internal care unit (ICU), and out of them, 100 (20%) patients developed acute kidney injury (AKI) while 400 (80%) patients did not develop AKI. It is also showed that 67 (67%) of those with AKI and 100 (25%) of those with no-AKI had baseline ventricular systolic dysfunction, left ventricular dysfunction (LVD), right ventricular dysfunction (RVD), and biventricular dysfunction (BiVD)presented in 23 (23%), 16 (16%), and 28 (28%) patients of AKI group, respectively, and presented in 60 (15%), 30 (7.50%), and 10 (2.50%) patients, respectively, in patients without acute kidney injury (AKI) Short conclusion Our study revealed that AKI has highest incidence in patient with biventricular dysfunction followed by left ventricular dysfunction and lastly those with right ventricular dysfunction.


Author(s):  
Carolina Shalini Singarayar ◽  
Foo Siew Hui ◽  
Nicholas Cheong ◽  
Goay Swee En

Summary Thyrotoxicosis is associated with cardiac dysfunction; more commonly, left ventricular dysfunction. However, in recent years, there have been more cases reported on right ventricular dysfunction, often associated with pulmonary hypertension in patients with thyrotoxicosis. Three cases of thyrotoxicosis associated with right ventricular dysfunction were presented. A total of 25 other cases of thyrotoxicosis associated with right ventricular dysfunction published from 1994 to 2017 were reviewed along with the present 3 cases. The mean age was 45 years. Most (82%) of the cases were newly diagnosed thyrotoxicosis. There was a preponderance of female gender (71%) and Graves’ disease (86%) as the underlying aetiology. Common presenting features included dyspnoea, fatigue and ankle oedema. Atrial fibrillation was reported in 50% of the cases. The echocardiography for almost all cases revealed dilated right atrial and or ventricular chambers with elevated pulmonary artery pressure. The abnormal echocardiographic parameters were resolved in most cases after rendering the patients euthyroid. Right ventricular dysfunction and pulmonary hypertension are not well-recognized complications of thyrotoxicosis. They are life-threatening conditions that can be reversed with early recognition and treatment of thyrotoxicosis. Signs and symptoms of right ventricular dysfunction should be sought in all patients with newly diagnosed thyrotoxicosis, and prompt restoration of euthyroidism is warranted in affected patients before the development of overt right heart failure. Learning points: Thyrotoxicosis is associated with right ventricular dysfunction and pulmonary hypertension apart from left ventricular dysfunction described in typical thyrotoxic cardiomyopathy. Symptoms and signs of right ventricular dysfunction and pulmonary hypertension should be sought in all patients with newly diagnosed thyrotoxicosis. Thyrotoxicosis should be considered in all cases of right ventricular dysfunction or pulmonary hypertension not readily explained by other causes. Prompt restoration of euthyroidism is warranted in patients with thyrotoxicosis complicated by right ventricular dysfunction with or without pulmonary hypertension to allow timely resolution of the abnormal cardiac parameters before development of overt right heart failure.


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