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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