LEFT VENTRICULAR DYSFUNCTION FOLLOWING NEONATAL PULMONARY VALVE BALLOON DILATION FOR PULMONARY ATRESIA OR CRITICAL PULMONARY STENOSIS

AbstractIn this study, we summarise a case of a myofibroma causing mid-aortic syndrome due to obstruction of the distal thoracic and abdominal aorta leading to severe left ventricular dysfunction. The patient was managed with percutaneous intervention via balloon dilation and stent placement. On follow-up, the patient has normalisation of ventricular function, is off anti-hypertensives, and is being monitored for re-stenosis.

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

Journal of the American Heart Association ◽

10.1161/jaha.120.019713 ◽

2021 ◽

Author(s):

Anjali Chelliah ◽

Anita J. Moon‐Grady ◽

Shabnam Peyvandi ◽

Joanne S. Chiu ◽

James E. Bost ◽

...

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Left Ventricular Dysfunction ◽

Ventricular Dysfunction ◽

Pulmonary Valve ◽

Right Ventricular Dysfunction ◽

Left Ventricular ◽

Absent Pulmonary Valve ◽

Intention To Treat ◽

Overall Mortality

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.

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Additional mechanism for left ventricular dysfunction: chronic pulmonary regurgitation decreases left ventricular preload in patients with tetralogy of Fallot

Cardiology in the Young ◽

10.1017/s1047951117001457 ◽

2017 ◽

Vol 28 (2) ◽

pp. 208-213 ◽

Cited By ~ 1

Author(s):

Pekka Ylitalo ◽

Eero Jokinen ◽

Kirsi Lauerma ◽

Miia Holmström ◽

Olli M. Pitkänen-Argillander

Keyword(s):

Left Ventricle ◽

Right Ventricular ◽

Tetralogy Of Fallot ◽

Left Ventricular Dysfunction ◽

Ventricular Dysfunction ◽

Pulmonary Valve ◽

Pulmonary Regurgitation ◽

Left Ventricular ◽

Valve Annulus ◽

Left Ventricular Preload

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.

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