The abnormal origin of the left pulmonary artery (LPA) from the ascending aorta is a rare cardiac condition that is often associated with several other congenital defects. In this paper, we report the case of an infant who presented with recurrent infections and was prenatally diagnosed with tetralogy of Fallot (TOF). During echocardiography, various other cardiac defects such as ventricular septal defects (VSD), pulmonary stenosis (PS), and dilated right heart chambers were identified. Furthermore, cardiac catheterization revealed an anomalous origin of the LPA arising from the aorta associated with a narrow pulmonary annulus. Due to both conditions sharing a similar embryological course, the condition is commonly associated with a conotruncal defect known as DiGeorge syndrome. Together, the overall combination of cardiac anomalies is both unusual and unique. This case study explains the clinical associations, embryological origin, and surgical management of this condition in an infant.
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http://dx.doi.org/10.7759/cureus.62916 | DOI Listing |
Cardiovasc Diagn Ther
December 2024
Department of Cardiology, St. Luke's International Hospital, Tokyo, Japan.
Tetralogy of Fallot (TOF) is a condition that often leads to long-term enlargement of the aortic root in after surgery. The aortic dilation is believed to be caused by histological abnormalities of the aortic media and the hemodynamic characteristics of increased aortic flow, compared to pulmonary flow. Severe cyanosis, severe right ventricular outflow tract (RVOT) obstruction, older age at repair, a larger aortic size at the time of repair, and a history of an aortopulmonary shunt parameters related to long-standing volume overload of the aortic root were the reported risk factors.
View Article and Find Full Text PDFBiomolecules
December 2024
Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", 34137 Trieste, Italy.
Glycosylphosphatidylinositol (GPI) biosynthesis defect 11 (GPIBD11), part of the heterogeneous group of congenital disorders of glycosylation, is caused by biallelic pathogenic variants in . This rare disorder has previously been described in only 12 patients. We report four novel patients: two sib fetuses with congenital anomalies affecting several organs, including the heart; a living girl with tetralogy of Fallot, global developmental delay, behavioral abnormalities, and atypic electroencephalography (EEG) without epilepsy; a girl with early-onset, treatment-resistant seizures, developmental regression, and recurrent infections, that ultimately passed away prematurely due to pneumonia.
View Article and Find Full Text PDFAnn Noninvasive Electrocardiol
January 2025
Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Background: Electrocardiograms (EKGs) are routinely performed in pregnant patients with pre-existing cardiovascular disease. However, in pregnant patients with congenital heart disease (CHD), EKG changes during gestation have not been explored.
Methods: We performed a retrospective study of pregnant patients with CHD enrolled in the STORCC initiative.
Perfusion
January 2025
Department of Pediatric and Congenital Heart Surgery, Onassis Cardiac Surgery Center, Athens, Greece.
Objectives: Compare outcomes of Del Nido (DN) versus conventional blood cardioplegia (BC) in the surgical repair of Tetralogy of Fallot (ToF).
Methods: Medical databases were searched to identify relevant clinical trials. Meta-analysis was conducted for primary (cardiopulmonary bypass [CPB] and aortic cross-clamp [ACC] times, hospital and intensive care unit [ICU] length of stay [LOS], mechanical ventilation time) and secondary (adverse events, lactate levels, volume of additional cardioplegia) endpoints.
Sci Rep
January 2025
Department of Ultrasound, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, 362000, Fujian, China.
Amniotic fluid (AF)-derived exosomal miRNA have been explored as potential contributors to the pathogenesis of Tetralogy of Fallot (TOF). This study aimed to investigate the expression profiles of AF-derived exosomal miRNAs and their potential contribution to TOF development. Exosomes were isolated from AF samples obtained from pregnant women carrying fetuses diagnosed with TOF.
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