- Congenital heart disease in adults: Assessmentof functional capacity using cardiopulmonary exercise testing. [Journal Article]
- RPRev Port Cardiol 2018 May 15
- CONCLUSIONS: Across the spectrum of CHD, cardiac shunts (particularly in those with Eisenmenger syndrome) and complex defects were associated with lower functional capacity and attenuated chronotropic response to exercise.
- Bordetella pertussis in children hospitalized with a respiratory infection: clinical characteristics and pathogen detection in household contacts. [Journal Article]
- BRBMC Res Notes 2018 May 18; 11(1):318
- Describe the prevalence of Bordetella pertussis via PCR in children under 5 years old hospitalized as probable cases of pertussis and report the most common clinical features among them.
Describe the prevalence of Bordetella pertussis via PCR in children under 5 years old hospitalized as probable cases of pertussis and report the most common clinical features among them.
- Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly. [Journal Article]
- AOActa Obstet Gynecol Scand 2018 May 16
- CONCLUSIONS: Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised in order to minimize maternal cardiac risk and select the appropriate mode of delivery. This article is protected by copyright. All rights reserved.
- [Cyanosis in 14-year-old patient. Methemoglobinemia: case report]. [Journal Article]
- AAArch Argent Pediatr 2018 06 01; 116(3):e429-e432
- The bluish coloration of skin and mucous membranes, called as cyanosis, could be explained by high reduced hemoglobin in the capillaries, or the presence of elevated methemoglobin concentration. It i...
The bluish coloration of skin and mucous membranes, called as cyanosis, could be explained by high reduced hemoglobin in the capillaries, or the presence of elevated methemoglobin concentration. It is important to think of methemoglobinemia as a differential diagnosis in a cyanotic patient who does not respond to oxygen administration once cardiorespiratory causes are discarded; since it requires other diagnostic methods and specific treatment. We described a case of cyanosis in a fourteen-year-old adolescent with probable congenital methemoglobinemia. We discussed their probable causes, clinic presentation, diagnosis and treatment.
- Iatrogenic atrial septal defect with right-to-left shunt following atrial fibrillation ablation in a patient with arrhythmogenic right ventricular cardiomyopathy. [Journal Article]
- HCHeartRhythm Case Rep 2018; 4(4):159-162
- The upper pouch in oesophageal atresia shows proportional growth during late fetal life. [Journal Article]
- APActa Paediatr 2018 May 12
- Oesophageal atresia with trachea-oesophageal fistula is a rare foregut malformation that requires surgery soon after birth. Prenatal ultrasound diagnosis is based on the presence of polyhydramnios, a...
Oesophageal atresia with trachea-oesophageal fistula is a rare foregut malformation that requires surgery soon after birth. Prenatal ultrasound diagnosis is based on the presence of polyhydramnios, a small or non-visible fetal stomach and the blind ending oesophagus, called the upper pouch (1). Neonates present with salivation, coughing, choking and attacks of cyanosis. A diagnosis of oesophageal atresia is confirmed by inserting a nasogastric tube and a thoraco-abdominal X-ray. This article is protected by copyright. All rights reserved.
- Congenital methemoglobinemia misdiagnosed as polycythemia vera: Case report and review of literature. [Journal Article]
- HRHematol Rep 2018 Mar 02; 10(1):7221
- Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, h...
Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis. Clinical cyanosis and lowoxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia. Arterial blood gas revealed a methemoglobin level of 38% (normal: 0-1.5%). Cytochrome B5 reductase (Methemoglobin reductase B) was deficient at level of <2.6 U/g Hb) (normal: 6.6-13.3), consistent with methemoglobin reductase (cytochrome b5) deficiency and hence the diagnosis of congenital methemoglobinemia was established. The role of Imatinib in provoking methemoglobinemia is questionable and association between Imatinib and methemoglobinemia never described before. In our case, there were no other offending drugs in aggravating the patients' symptoms and cyanosis. The patient started on Vitamin C 500 mg once daily for which he responded well with less cyanosis and significant reduction of methemoglobin level. Congenital methemoglobinemia is a rare underreported hemoglobin disease and often clinically missed. Upon extensive review of English literature for cases of congenital methemoglobinemia due to deficiency of cytochrome b5 reductase, we found 23 cases diagnosed as type I (including the case reported here). 17 cases (~74%) of type I and 6 cases (27%) of type II. There is male predominance 73% versus 26% in females. Almost half of reported cases 12 cases (52%) are Indian, 2 Japanese, 3 English, 2 Arabic, one case Spanish and one case Italian. For type I, the median calculated age is 31 years with cyanosis and shortness of breath being the most common sign and symptoms. For type II: Six cases were reported in English literature, all in pediatric age group with median calculated age at presentation is 6 years with neurologic manifestations and mental retardation are the most common type II associated symptoms. Due to lack of systematic epidemiological studies, congenital methemoglobinemia is under diagnosed as it is under investigated and usually overlooked especially when presenting in adulthood and in absence of obvious acquired agents.
- Successful repair of neonatal tricuspid regurgitation due to chordae rupture. [Journal Article]
- JTJ Thorac Dis 2018; 10(3):E186-E188
- Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgi...
Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.
- Association of Raynaud's phenomenon with a polymorphism in the NOS1 gene. [Journal Article]
- PlosPLoS One 2018; 13(4):e0196279
- CONCLUSIONS: Results indicate that RP is associated with variation in gene NOS1. This finding may be related to the observation that the significant SNP in NOS1 is known to exhibit functional influence on the gene expression.
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- Assessment of Potential Renal Dysfunction in Patients with Congenital Heart Disease after Biventricular Repair. [Journal Article]
- KCKorean Circ J 2018; 48(5):418-426
- CONCLUSIONS: Increased UACR was noted in 43% of patients. In patients with UACR ≥10 mg/gCr, right heart system abnormality was observed, and several patients had cyanosis before radical treatment. Measurement for UACR may be able to detect renal dysfunction early in the postoperative remote period.