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Multimedia Manual of Cardiothoracic... Aug 2023Total anomalous pulmonary venous return due to septum primum malposition is a poorly understood condition despite being very common in left atrial isomerism or...
Total anomalous pulmonary venous return due to septum primum malposition is a poorly understood condition despite being very common in left atrial isomerism or polysplenia syndrome. Due to the leftward displacement of the septum primum, either the two right pulmonary veins or all four pulmonary veins can drain abnormally into the right atrium, despite their correct position. In other words, the four pulmonary veins (or the two right pulmonary veins), looking from outside the heart, return at the back of the atrium in the normal position. Nevertheless, from the inside of the heart, two or all four pulmonary veins drain into the right atrium due to the leftward displacement of the septum primum. As an example, we report a 5-month-old patient with severe malposition of the septum primum and consequent total anomalous pulmonary venous drainage into the right atrium. The patient underwent surgical correction with resection of the malpositioned septum primum and reconstruction of a normal interatrial septation with a pericardial patch.
Topics: Humans; Infant; Heterotaxy Syndrome; Heart Septal Defects, Atrial; Scimitar Syndrome; Heart Atria; Pulmonary Veins
PubMed: 37578040
DOI: 10.1510/mmcts.2023.035 -
Cureus Jul 2023This study describes the successful management of gangrenous pneumococcal infection in an infant with polysplenia, a rare congenital malformation characterized by...
This study describes the successful management of gangrenous pneumococcal infection in an infant with polysplenia, a rare congenital malformation characterized by multiple aberrant nodules of splenic tissue. The patient, a three-month-old girl with congenital heart defects and incomplete vaccination, presented with fever, irritability, and oxygen desaturation, followed by erythematous skin changes. The diagnosis revealed sepsis caused by , accompanied by extensive gangrenous skin lesions and signs of disseminated intravascular coagulation. Polysplenia was incidentally discovered during radiological investigation. Aggressive resuscitation measures and prolonged antibiotic administration led to significant improvement, including regression of skin lesions. This case emphasizes the importance of timely immunization and parental awareness for infants with spleen-related congenital malformations. Screening for undiagnosed malformative defects in congenital heart disease patients can aid in early detection and prevention of life-threatening complications. The successful management demonstrates the critical role of pediatric intensive care units in treating severe systemic infections.
PubMed: 37546058
DOI: 10.7759/cureus.41390 -
Asian Journal of Surgery Dec 2023
Topics: Humans; Heterotaxy Syndrome; Renal Veins; Tomography, X-Ray Computed
PubMed: 37541886
DOI: 10.1016/j.asjsur.2023.07.115 -
Frontiers in Cardiovascular Medicine 2023To explore the diagnostic clues and abnormality spectrum of heterotaxy syndrome by prenatal ultrasonography and postnatal verification.
BACKGROUND
To explore the diagnostic clues and abnormality spectrum of heterotaxy syndrome by prenatal ultrasonography and postnatal verification.
METHODS
The prenatal ultrasonic data of 88 heterotaxy syndrome fetuses were analyzed retrospectively as left isomerism (LI) and right isomerism (RI). Prenatal ultrasound compared with the anatomical casting of the fetal body after labor induction, and the confirmatory postnatal diagnosis after delivery.
RESULTS
Fetal LI showed typical malformations of gastric vesicles on different sides from the heart, absence of hepatic segment of the inferior vena cava (IVC), abdominal aorta (AO) parallel with the azygos vein (AV), bilateral left bronchus, bilateral left atrial appendages, and polysplenia; intracardiac malformations of AV septal defects (AVSD), single atrium (SA), left ventricular outflow tract obstruction (LVOTO), and double-outlet right ventricle (DORV); and cardiac conduction abnormalities of sinus bradycardia and AV blockage. Fetal RI reported typical malformations of gastric vesicles on different sides from the heart, juxtaposition of the IVC with AO, anomalous pulmonary venous connection (APVC), asplenia, and bilateral right atrial appendages; intracardiac malformations of AVSD, SA, single ventricle, pulmonary atresia and stenosis, and DORV. The postnatal verification revealed 3 malformations misdiagnoses and 4 malformations missed diagnoses in LI fetuses and 10 misdiagnoses and 8 missed diagnoses in RI fetuses.
CONCLUSIONS
The proposed five-step prenatal ultrasonography has an important diagnostic value for the identification and classification of heterotaxy syndrome. The different sides of gastric vesicles and cardiac apex are important diagnostic clues for heterotaxy syndrome, featuring disconnected or hypoplastic IVC, typical complex cardiac malformation, and atrioventricular block in fetal LI, and shown APVC, juxtaposition of IVC and AO, and intracardiac malformations such as AVSD, DORV, and LVOTO in fetal RI.
PubMed: 37485264
DOI: 10.3389/fcvm.2023.1195191 -
Clinical Case Reports Jun 2023A 26-year-old male patient admitted to the hospital ward with experience of repetitive syncopes for a year. The patient was diagnosed with sick sinus syndrome. The aim...
KEY CLINICAL MESSAGE
A 26-year-old male patient admitted to the hospital ward with experience of repetitive syncopes for a year. The patient was diagnosed with sick sinus syndrome. The aim of this clinical report is to highlight the variability of anatomical findings associated with polysplenia pattern.
ABSTRACT
This case report presents a 26-year-old male patient who presented to the medical ward with a complaint of repeating blackouts for a year. The patient was then diagnosed with sick sinus syndrome, and further investigations revealed left isomerism, polysplenia, and no congenital heart defects. Holter monitoring, ultrasonography, electrocardiography, and computed tomography were used to confirm the diagnosis. The patient underwent DDDR pacemaker implantation for the treatment of SA node dysfunction. The report highlights the variability of anatomical findings associated with polysplenia pattern and the various types of heartbeat disruptions that may occur in the atrial appendages of the left side isomerism.
PubMed: 37334338
DOI: 10.1002/ccr3.7511 -
Cureus May 2023Polysplenia syndrome is a rare congenital anomaly characterized by multiple spleens and associated organ anomalies, which can present with serious complications such as...
Polysplenia syndrome is a rare congenital anomaly characterized by multiple spleens and associated organ anomalies, which can present with serious complications such as splenic infarction. Diagnosis and management of the disorder can be challenging due to the presence of associated anomalies and the condition is often diagnosed incidentally. We report a case of a six-year-old girl with no significant medical history who presented to the emergency department with fever, abdominal pain, and vomiting. Physical examination and laboratory investigations showed leukocytosis, anemia, and elevated levels of C-reactive protein. A computed tomography scan revealed splenic infarction with polysplenia syndrome. The patient received intravenous antibiotics and pain management and was closely monitored for complications such as sepsis. Early diagnosis and appropriate management are essential to prevent complications, and close monitoring and follow-up are necessary for long-term management.
PubMed: 37284391
DOI: 10.7759/cureus.38589 -
Journal of the West African College of... 2023Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with...
Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with various congenital anomalies and non-syndromic (isolated anomaly). We present a case of syndromic BA associated with polysplenia and intestinal malrotation, discovered incidentally during the Kasai procedure. The small intestine was found to be non-rotated with the duodenojejunal junction to the right of the vertebral column. The presence of accessory spleens was noted. Kasai portoenterostomy and Ladd's procedure were performed. The patient had an uneventful postoperative course with the passage of cholic stool from the third postoperative day. At the seventh-month follow-up, the stool remained cholic. A multidisciplinary approach in the care of babies with BA and long-term follow-up is crucial for a successful outcome.
PubMed: 36923807
DOI: 10.4103/jwas.jwas_195_22 -
Cureus Feb 2023Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional...
Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date.
PubMed: 36909095
DOI: 10.7759/cureus.34689 -
Radiology Case Reports May 2023The heterotaxy syndrome is a type of syndrome that involves multiple visceral abnormalities, vascular ones and associated with left isomerism. Malformation of...
The heterotaxy syndrome is a type of syndrome that involves multiple visceral abnormalities, vascular ones and associated with left isomerism. Malformation of gastroenterologic system includes polysplenia (segmented spleen or multiple splenules), agenesis (partial or complete) of the dorsal pancreas and anomalous of the inferior vena cava implantation. Here, we describe and show the anatomy of a patient with left side inferior vena cava, situs ambiguous (complete common mesentery), polysplenia, and short pancreas. We also discuss about the embryologic process and the implications of these anomalies during gynecologic, digestive, and liver surgeries.
PubMed: 36895889
DOI: 10.1016/j.radcr.2023.02.005