scholarly journals Transposition of Great Arteries

2021 ◽  
Author(s):  
Rita Prasad Verma
Keyword(s):  
Fetal Echocardiography ◽  
Arterial Switch Operation ◽  
Cardiac Anomaly ◽  
First Year ◽  
Term Outcome ◽  
Hemodynamic Pattern ◽  
Switch Operation ◽  
The Right ◽  
First Year Of Life

TGA is the commonest complex congenital cyanotic cardiac anomaly occurring during the first week of life. It is characterized by the unusual anomaly of ventriculoarterial discordance, with the aorta (A) originating from the right ventricle (RV) and the pulmonary artery (PA) from the left ventricle (LV). In the common Dextro form (DTGA), A is abnormally located to the right, anterior, and inferior of PA. The anatomic configuration results in the lethal hemodynamic pattern of 2 independent and parallel running circulatory circuits, which mandates creating a conduit to ensure the mixing of oxygenated and deoxygenated blood for survival. In the rare Levo form (LTGA), the aorta is placed anterior and to the left of PA with ventricular inversion. TGA is well tolerated in the fetus and is challenging to diagnose by fetal echocardiography unless the outflow tracts are specifically visualized. Postnatally the typical findings of murmur and cyanosis vary according to the associated cardiac defects and the degree of intercirculatory mixing. The arterial switch operation (ASO), which involves establishing ventriculoarterial concordance, is the standard surgical repair of D-TGA and has replaced the atrial switch procedures due to its superior long-term outcomes. The Rastelli procedure is used for complex DTGA cases. DTGA has a 90% mortality rate in the first year of life if untreated, while over 95% survive for 5 to 25 years after surgery. Post-surgical course may be complicated and require surgical revisions. The long-term outcome is associated with normal or mild to moderate neurodevelopmental disabilities, depending upon the type, complexity, and course of the disease. Expert follow-up of the patients into adulthood is an integral part of the management of TGA for best outcomes.

Muenke syndrome: long-term outcome of a syndrome-specific treatment protocol

2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  
Keyword(s):  
Treatment Protocol ◽  
First Year ◽  
Ventricular Size ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Skull Growth ◽  
Muenke Syndrome ◽  
First Year Of Life

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

Long-term outcome after surgical repair of transposition of the great arteries: atrial versus arterial switch operation

2010 ◽  
Vol 58 (S 01) ◽  
Author(s):  
H Görler ◽  
D Böthig ◽  
M Ono ◽  
M Westhoff-Bleck ◽  
A Haverich ◽  
...  
Keyword(s):  
Surgical Repair ◽  
Arterial Switch Operation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Arterial Switch ◽  
Switch Operation

Anterosuperior Diverticula of the Right Ventricle: Morphological Spectrum and Long-Term Outcome of a Distinct Cardiac Anomaly

2014 ◽  
Vol 35 (6) ◽  
pp. 983-989 ◽  
Author(s):  
Gesa Wiegand ◽  
Ralf Rauch ◽  
Helmut Singer ◽  
Andreas Koch ◽  
Michael Hofbeck
Keyword(s):  
Right Ventricle ◽  
Cardiac Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
The Right

Functional assessment of the heart after corrective surgery for complete transposition

1991 ◽  
Vol 1 (1) ◽  
pp. 84-90 ◽  
Author(s):  
Andrew N. Redington
Keyword(s):  
Right Ventricle ◽  
Functional Assessment ◽  
Functional Performance ◽  
Arterial Switch Operation ◽  
Corrective Surgery ◽  
Arterial Switch ◽  
Complete Transposition ◽  
Switch Operation ◽  
The Right

SummaryThe justification for the introduction of the arterial switch procedure was based, primarily, on concern regarding the long-term ability of the right ventricle to perform as the systemic pumping chamber. In this article, the functional performance of both the systemic and pulmonary ventricles after atrial redirection procedures and the arterial switch operation will be discussed.

scholarly journals Long-Term Outcome of Arterial Switch Operation Conversion After Failed Senning/Mustard Procedure

2016 ◽  
Vol 102 (5) ◽  
pp. 1573-1579 ◽  
Author(s):  
Takuya Maeda ◽  
Takahiko Sakamoto ◽  
Mitsugi Nagashima ◽  
Takeshi Hiramatsu ◽  
Kenji Yamazaki
Keyword(s):  
Arterial Switch Operation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Arterial Switch ◽  
Switch Operation ◽  
Mustard Procedure

Long-term Outcome and Quality of Life after Arterial Switch Operation: A Prospective Study with a Historical Comparison

2013 ◽  
Vol 8 (3) ◽  
pp. 203-210 ◽  
Author(s):  
Titia P.E. Ruys ◽  
Annemien E. van der Bosch ◽  
Judith A.A.E. Cuypers ◽  
Maarten Witsenburg ◽  
Willem A. Helbing ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Prospective Study ◽  
Arterial Switch Operation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Historical Comparison ◽  
Switch Operation ◽  
A Prospective Study

scholarly journals 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: One disease- many faces

2020 ◽  
Author(s):  
Sarah Catharina Grünert ◽  
Jörn Oliver Sass
Keyword(s):  
Autosomal Recessive ◽  
Coenzyme A ◽  
Autosomal Recessive Disorder ◽  
First Year ◽  
Metabolic Decompensation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Lyase Deficiency ◽  
First Year Of Life

Abstract Background 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMGCLD) is an autosomal recessive disorder of ketogenesis and leucine degradation due to mutations in HMGCL . Method We performed a systematic literature search to identify all published cases. 211 patients of whom relevant clinical data were available were included in this analysis. Clinical course, biochemical findings and mutation data are highlighted and discussed. An overview on all published HMGCL variants is provided. Results More than 95 % of patients presented with acute metabolic decompensation. Most patients manifested within the first year of life, 42.4 % already neonatally. Very few individuals remained asymptomatic. The neurologic long-term outcome was favorable with 62.6 % of patients showing normal development. Conclusion This comprehensive data analysis provides a systematic overview on all published cases with HMGCLD including a list of all known HMGCL mutations.

scholarly journals Motor Development of Premature Infants Born between 32 and 34 Weeks

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
S. A. Prins ◽  
J. S. von Lindern ◽  
S. van Dijk ◽  
F. G. A. Versteegh
Keyword(s):  
Motor Development ◽  
Late Preterm ◽  
First Year ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Normal Outcome ◽  
Movement Assessment ◽  
Preterm Born ◽  
First Year Of Life

Little is known about motor development in late preterm born infants. Our objective was to determine long-term outcome of motor skills of infants born between 32 and 34 weeks. All infants were assessed at corrected ages of 3 and 9 months, using the Alberta Infant Motor Scale. At corrected ages of 4 years, the Movement Assessment Battery for Children was done. Seventy infants were seen at 4 years of age (median of 3 assessments per infant). Abnormal assessment at 3 or 9 months of age resulted in normal outcome in almost 80% at 4 years. On the other hand, a normal outcome in the first year of life resulted in an abnormal outcome at 4 years in 10% of the infants. Our results suggest that long-term followup of these late preterm born infants is necessary, as the assessments in the first year do not predict the long-term outcome.

scholarly journals 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: One disease- many faces

2019 ◽  
Author(s):  
Sarah Catharina Grünert ◽  
Jörn Oliver Sass
Keyword(s):  
Autosomal Recessive ◽  
Coenzyme A ◽  
Autosomal Recessive Disorder ◽  
First Year ◽  
Metabolic Decompensation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Lyase Deficiency ◽  
First Year Of Life

Abstract Background 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMGCLD) is an autosomal recessive disorder of ketogenesis and leucine degradation due to mutations in HMGCL .Method We performed a systematic literature search to identify all published cases. 211 patients of whom relevant clinical data were available were included in this analysis. Clinical course, biochemical findings and mutation data are highlighted and discussed. An overview on all published HMGCL variants is provided.Results More than 95% of patients presented with acute metabolic decompensation. Most patients manifested within the first year of life, 42.4% already neonatally. Very few individuals remained asymptomatic. The neurologic long-term outcome was favorable with 62.6% of patients showing normal development.Conclusion This comprehensive data analysis provides a systematic overview on all published cases with HMGCLD including a list of all known HMGCL mutations.

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