UNASSIGNED: Methemoglobin is an altered state of hemoglobin where iron in hemoglobin is oxidized and incapable of binding oxygen; leading to complications such as cyanosis, dyspnea, headache, and heart failure. Methemoglobinemia can be congenital or acquired. Congenital methemoglobinemia is a rare disease and its worldwide incidence is unclear. We recently encountered the first documented case of congenital methemoglobinemia at our institution, necessitating perioperative care.
UNASSIGNED: In the present case, a 22-year-old man with congenital methemoglobinemia underwent general anesthesia for dental extraction. The surgeon was informed to avoid local anesthetics and oxygenation was performed with FiO2 of 1.0. Arterial blood gas analysis showed a PH of 7.337, PaO2 of 302 mm Hg, PaCO2 of 44 mm Hg, oxyhemoglobin level of 63.4%, and methemoglobin level of 37.8%. The patient had a stable course. No methylene blue therapy was required, although cyanosis was observed during surgery.
UNASSIGNED: In summary, though rare, congenital methemoglobinemia poses fatal risks during surgery. Its management involves preoperative recognition and optimization, oxygenation status, multidisciplinary care, avoiding precipitating or oxidizing agents, discussing treatment options, maintaining cardiopulmonary stability, and ensuring perioperative safety measures with the medical team.

BACKGROUND: Tracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature.
METHODS: This study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature.
RESULTS: We identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation.
CONCLUSIONS: Esophageal intubation may be a life-sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.

BACKGROUND: Ethnic inequalities in maternal and neonatal health in the UK are well documented. Concerns exist regarding the use of skin colour in neonatal assessments. Healthcare professionals should be trained to recognise symptoms of diverse skin tones, and comprehensive, and inclusive guidance is necessary for the safe assessment of all infants. Disparities in healthcare provision have been emphasised during the COVID-19 pandemic, and additional research is needed to determine whether such policies adequately address ethnic minority neonates.
METHODS: A desktop search included searches of guidance produced for the United Kingdom (UK). Further searches of the Cochrane and World Health Organization (WHO) were used to identify any international guidance applicable in the UK context.
RESULTS: Several policies and one training resource used descriptors \'pink,\' \'pale,\' \'pallor,\' and \'blue\' about neonatal skin and mucous membrane colour. No policies provided specific guidance on how these colour descriptors may appear in neonates with different skin pigmentation. Only the NICE guidance and HEE e-learning resource acknowledged the challenges of assessing jaundice in infants with diverse skin tones, while another guideline noted differences in the accuracy of bilirubin measurements for the assessment of jaundice. Three policies and one training resource advised against relying on visual observation of skin colour when diagnosing neonatal conditions. The training resource included images of ethnic minority neonates, although most images included white infants.
CONCLUSIONS: Inadequate consideration of ethnicity in UK policy and training perpetuates disparities, leading to inaccurate assessments. A review is needed for inclusivity in neonatal care, regardless of skin pigmentation.

BACKGROUND: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as \"pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level.\" It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES.
METHODS: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength.
CONCLUSIONS: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.

UNASSIGNED: Neonatal cyanosis is a clinical manifestation of hypoxemia and is usually pathological. Persistent right venous valve (PRVV) is a rare cause of cyanosis in newborns and can cause prenatal abnormalities, the clinical significance of which varies depending on the severity of the abnormality. There have been few reports on the intrauterine detection of these abnormalities and their follow-up during infancy. Here, we report a case of PRVV causing supravalvular tricuspid valve (TV) obstruction and secondary right ventricle (RV) hypoplasia. This case is unique in terms of its early prenatal detection, distinct cardiac anomalies, and successful surgery that reversed the symptoms, and the findings offer insights into the diagnosis and management of such rare cardiac conditions.
UNASSIGNED: We report a case of a newborn diagnosed with PRVV at 31 weeks of gestation at our center. There was no underlying family history of congenital heart disease. Prenatal sonography identified an echogenic membrane in the right atrium, suggesting TV obstruction and subsequent RV hypoplasia. After birth, the neonate suffered hypoxia with decreased arterial oxygen saturation (SaO2). Minimally invasive surgery successfully corrected the membrane. Postoperative SaO2 improved immediately. Three months later, follow-up echocardiography revealed normalized TV and RV dimensions. The patient demonstrated steady progress without any complications. We also reviewed previous cases of PRVV before and after birth and summarized the sonographic and clinically relevant features.
UNASSIGNED: Although PRVV is typically considered as a benign structure, it may lead to significant clinical complications, particularly in fetuses and neonates. The precise identification of its variant forms and related flow patterns is crucial to inform decisions regarding patient management.

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking \"Budd-Chiari syndrome\" and \"hepatopulmonary syndrome\" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.
目的： 总结Budd-Chiari综合征合并肝肺综合征（HPS）患儿的临床特征及预后。 方法： 回顾性分析2016年12月郑州大学第一附属医院儿科收治的1例Budd-Chiari综合征合并HPS患儿的临床资料。以“Budd-Chiari综合征”“肝肺综合征”“Budd-Chiari syndrome”“hepatopulmonary syndrome”为关键词分别在中国知网、万方数据库、中国生物医学文献数据库、PubMed数据库进行检索（建库至2023年7月），结合本例资料，总结Budd-Chiari综合征合并HPS患儿（<18岁）的临床特征、诊疗经过及预后。 结果： 患儿，男，13岁，因“发绀、活动后胸闷6个月，皮肤黄染1周”入院。入院体格检查可见全身皮肤黏膜、巩膜轻度黄染，口唇、眼周、四肢末端发绀。辅助检查可见肝功能及动脉血气分析异常，总胆红素53 μmol/L，直接胆红素14 μmol/L，间接胆红素39 μmol/L；氧分压54 mmHg（1 mmHg=0.133 kPa），二氧化碳分压31 mmHg，肺泡动脉血氧梯度57 mmHg；腹部CT静脉造影示Budd-Chiari综合征（肝静脉型）、肝硬化、门静脉高压；增强经胸超声心动图造影（CE-TTE）阳性。患儿经对症支持治疗后院外氧疗，随访至2023年3月，低氧血症无明显改善，日常活动受限。文献复习符合检索条件中文文献0篇，英文文献3篇，结合本例患儿共4例，3例首诊原因为呼吸困难、发绀或低氧血症，1例不详。2例因呼吸系统症状就诊诊断Budd-Chiari综合征合并HPS，2例HPS分别发生于Budd-Chiari综合征确诊1.5和8.0年。CE-TTE阳性2例，肺灌注显像阳性2例。2例患儿行肝移植，呼吸功能恢复良好；吸氧治疗1例，低氧血症无改善；等待肝移植1例。 结论： Budd-Chiari综合征合并HPS早期起病隐匿，临床表现以呼吸困难、发绀多见，早期行CE-TTE明确肺内血管扩张可减少误诊。肝移植有助于改善预后。.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease (CHD) in infants. The four components that constitute ToF are an overriding aorta over the crest of the interventricular septum, obstruction in the right ventricular outflow tract, right ventricular hypertrophy, and a typically large non-restricted ventricular septal defect. ToF may also be associated with other extracardiac abnormalities, including patent ductus arteriosus or multiple aortopulmonary collateral arteries, which can impact the patient\'s survival. Patients with unoperated ToF rarely reach adulthood, and it is extremely rare to discover undiagnosed ToF in individuals over 60 years old. In this report, we describe an unusual case of a woman with unrepaired ToF who survived until the age of 71. She was fairly asymptomatic until the 7th decade of her life and complained only of dyspnea on exertion. The patient declined corrective cardiac surgery and preferred conservative management.

BACKGROUND: Persistent pulmonary hypertension of the newborn manifesting with refractory and severe cyanosis is the consequence of high pulmonary vascular resistance causing extrapulmonary right-to-left shunt. Acidosis and hypoxemia produce pulmonary vasoconstriction. Persistent pulmonary hypertension of the newborn occurs due to numerous disorders and has been rarely reported as a manifestation of methylmalonic acidemia. We report a newborn with methylmalonic acidemia who presented with persistent pulmonary hypertension of the newborn.
METHODS: A 1-day-old Iranian girl presented with respiratory distress and refractory metabolic acidosis. She was born at 39 + 5 weeks gestational age with Apgar scores of 8 and 9 in the 1st and 5th minutes, respectively, and was in good condition up to 10 hours of life. After that, she presented with cyanosis, tachypnea, retraction, and hypotonia. Despite receiving oxygen, she had low oxygen saturation. Echocardiography revealed severe pulmonary hypertension and right-to-left shunt through patent ductus arteriosus and foramen ovale. Her acidosis worsened despite receiving full support and medical therapy. So, she was started on peritoneal dialysis. Unfortunately, she did not respond to treatment, and after she had died, biochemical tests confirmed methylmalonic acidemia.
CONCLUSIONS: Persistent pulmonary hypertension of the newborn is a very rare manifestation of methylmalonic acidemia. Severe inborn errors of metabolism may cause irreversible damage with adverse lifelong morbidity, and early diagnosis may help to prevent such complications. Furthermore, diagnosis of these disorders aids in prenatal diagnosis through the use of cultured amniocytes or chorionic villi to detect gene mutations, as well as biochemical analyses of amniotic fluid for subsequent pregnancies.

The development of veno-venous collaterals is an important and treatable cause of cyanosis in patients who had undergone partial cavo-pulmonary connection (PCPC) operations. Nevertheless, the literature on this complicated therapeutic option is sparse. Patients can present cyanosis either immediately after the operation (<30 days), which delays or hinders discharge from the intensive care unit or cyanosis may occur late: (>30 days and/or in another hospital admission), after the operation. Hence, transcatheter closure of veno-venous collaterals is the treatment of choice. Four patients were selected who showed cyanosis at variable durations after PCPC; the morphology of the collaterals and their hemodynamic effect was described and the strategy for closure of such abnormal vessels is suggested. Veno-venous collaterals described in our series were seen originating mainly or mostly from innominate vein angles. The drainage sites were either above the diaphragm into a cardiac structure: the coronary sinus (CS) and/or atria; or below the diaphragm into the inferior vena cava (IVC) or hepatic veins through the paravertebral venous system and/or the azygous system. It is stated in the literature that several types of devices and coils can be used to close the collaterals such as the Amplatzer vascular plugs (AVPs), Amplatzer duct occluder II (ADOII), non-detachable and detachable coils. In this clinical review, the technical details that determine device type and size are explained. The recent generations of hydrogel-coated coils were also used in this series of patients to close the difficult types of collaterals with better results. All described vessels were closed successfully, without any complications. The patients had a significant rise in their transcutaneous oxygen saturations and hence, a clear clinical benefit.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary complications are common and may contribute to both circulatory and pulmonary insufficiency, leading ultimately to Fontan failure. These complications include restrictive lung disease, sleep abnormalities, plastic bronchitis, and cyanosis. Cyanosis post-Fontan procedure can be attributed to multiple causes including systemic to pulmonary venous collateral channels and pulmonary arteriovenous malformations. This review presents the unique cardiopulmonary interactions in the Fontan circulation. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this growing patient population. Interventions focused on improving pulmonary function including inspiratory muscle training and endurance training have shown a promising effect post-Fontan procedure.