Partial defect of the pericardium combined with bronchogenic cyst is a very rare congenital anomaly. We describe the case of a 32-year-old man with a partial defect of the left pericardium and a bronchogenic cyst arising from the border of the pericardial defect. The cyst was successfully resected with the harmonic scalpel by three-port videothoracoscopic approach.

BACKGROUND HLHS (hypoplastic left heart syndrome) is a serious congenital heart defect. In neonates with congenital heart disease HLHS accounts for nearly 25% of neonatal deaths. The etiology of HLHS is unknown. At present we expect to have those cases diagnosed in the second trimester of pregnancy. AIM The main aim was to find the rate and outcome of the congenital heart defect--left heart h...

A survey conducted by cardiologists in Soweto, Johannesburg, provided an opportunity of assessing the frequency of congenital heart disease in black schoolchildren. Among 12,050 schoolchildren aged 2 to 18 years, 48 had a congenital heart defect, yielding a prevalence of 3.9 per 1000. Only in 2- to 6-year-old children did the prevalence exceed that of rheumatic heart disease. The distribution o...

Congenital indifference to pain (pain asymbolia) is a rare condition. Since Dearborn in 1931 described the first case, 51 cases have been reported in the English literature. Congenital auditory imperception as an isolated defect is equally uncommon. It is perhaps the least uncommon of the various types of congenital aphasia. We have recently studied a family in which two siblings showed an asso...

INTRODUCTION Congenital erythrocytosis is by definition present from birth. Patients frequently present in childhood or as young adults and a family history may be present. The erythrocytosis can be primary where there is a defect in the erythroid compartment of secondary where increased erythropoietin production produced due to the defect leads to an erythrocytosis. MATERIAL AND METHODS Prim...

(Abstracted from N Engl J Med 2021;385:107–118) Congenital diaphragmatic hernia (CDH) occurs in approximately 1 4000 births, and 85% of these cases have the defect on left side. The condition is associated with neonatal death due to respiratory failure pulmonary hypertension.

BACKGROUND Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. METHODS AND RESULTS A total of 1188 patients with adult c...

AIMS Pulmonary hypertension (PH) in asymptomatic patients is a rare cause of sudden death. This study aims to determine the incidence of this entity and raise awareness among pathologists. METHODS We retrospectively investigated 44 cases of sudden unexpected death in relation to PH in patients not on antihypertensive therapy. This is the largest pathological study reported. RESULTS We repor...

INTRODUCTION Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. CASE REPORT We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal d...

Congenital insensitivity to pain with anhidrosis is a rare disease of the nervous system which causes one to lose his/her feeling of pain. The disease is subtype four of hereditary sensory and autonomic neuropathy (HSAN IV) that results from NTRK1 gene defect. Direct sequencing was performed to screen NTRK1 for mutations. The result revealed a homozygous deletion of adenine on intron 14 that ma...