MyMedR

Tumour lysis syndrome in a neonate with transient abnormal myelopoiesis

Mohammad Khuzaini A, Mohd Baharudin JA, Md Fauzi A, Zulkeflee HA, Abdul Halim H, Mazli SK, et al.

J Neonatal Perinatal Med, 2024;17(2):269-273.

BACKGROUND: Tumor lysis syndrome (TLS) is an oncological emergency associated with hematological malignancies or highly proliferative solid tumors, commonly after chemotherapy. It is rarely associated with transient abnormal myelopoiesis.

OBSERVATION: We report a rare case of a neonate with transient abnormal myelopoiesis and tumor lysis syndrome, complicated with concomitant heart failure due to an underlying atrioventricular septal defect. Hyperhydration was contraindicated due to heart failure. The patient was managed conservatively with full recovery.

CONCLUSION: Tumor lysis syndrome should be suspected in neonates with transient abnormal myelopoiesis with electrolyte abnormalities. Treatment options should be considered carefully for their risks and benefits.

Matched MeSH terms: Heart Septal Defects, Atrial/complications

Beating the odds: a rare case of supracardiac total anomalous pulmonary venous return (TAPVR) in an adult patient

Talib N, Sayuti KA, Abdullah MS, Mohd Zain MR

BMJ Case Rep, 2018 Mar 05;2018.

PMID: 29507009 DOI: 10.1136/bcr-2017-221074

Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage. Cardiac CT confirmed supracardiac TAPVR, whereby all the pulmonary veins drain into the anomalous vein and finally to the superior vena cava. She remained asymptomatic and underwent a successful surgical repair.

Matched MeSH terms: Heart Septal Defects, Atrial/complications*

Atrial septal defect coexistent with Sjögren's syndrome

Ahmad F, Sadiq MA, Chee KH, Mahmood Zuhdi AS, Wan Ahmad WA

J Coll Physicians Surg Pak, 2014 Jun;24(6):441-3.

PMID: 24953923 DOI: 06.2014/JCPSP.441443

Pulmonary hypertension is frequently associated with atrial septal defect and various connective tissue disorders. This case describes a 74-year-old woman who presented with symptoms of heart failure and concomitant involvement of salivary glands and keratoconjunctivitis. An echocardiogram demonstrated ostium secundum atrial septal defect with left to right shunt and severe pulmonary hypertension. Laboratory investigations confirmed the diagnosis of Sjögren's syndrome (SS) with positive anti-nuclear factor and centromere SS-A/Ro pattern. Anti-Ro (SS-A) was found positive. Atrial septal defect was closed through transcatheter route with significant improvement in clinical outcome. This case report suggests a possible association of atrial septal defect with primary Sjögren's syndrome in an adult patient.

Matched MeSH terms: Heart Septal Defects, Atrial/complications*

Persistent valve of systemic venous sinus: a cause of neonatal cyanosis

Qureshi AU, Latiff HA, Sivalingam S

Cardiol Young, 2014 Aug;24(4):756-9.

PMID: 24016801 DOI: 10.1017/S1047951113001200

Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

Matched MeSH terms: Heart Septal Defects, Atrial/complications

Microscopical atrial amyloidosis in chronic heart disease

Looi LM

Histopathology, 1981 Nov;5(6):615-22.

PMID: 7319480

Nineteen out of 121 consecutive cardiac biopsies from 107 patients were found to contain amyloid deposits on routine Congo red screening. Seventeen were left atrial appendages removed during mitral valvotomy for chronic rheumatic mitral valve disease while the remaining two were right atrial appendages excised during surgical repair of atrial septal defects. The distribution of amyloid deposits within the atria and their tinctorial characteristics are described. The high prevalence of atrial amyloidosis observed could not be attributed to generalized or senile amyloidosis. The possibility that this is a distinctive localized form of amyloidosis secondary to chronic heart disease is discussed.

Matched MeSH terms: Heart Septal Defects, Atrial/complications

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