Cyanosis period: A key factor influencing exercise cardiac performance after Fontan procedure.

Background: Most patients with Fontan circulation struggle to increase cardiac output during exercise, affecting aerobic capacity and quality of life.

Aim: We sought to identify factors in the preFontan period and in management that influence their exercise cardiac performance.

Methods: We retrospectively collected anatomical, clinical and pre- and postFontan invasive haemodynamic data, including ventricular filling pressure in 57 consecutive Fontan patients who underwent cardiopulmonary exercise testing.

[Analyzing the reasons for and prevention of serious complications after general anesthesia in children with obstructive sleep apnea].

To explore the causes and preventive measures of respiratory arrest following general anesthesia in children with obstructive sleep apnea （OSA）, in order to enhance the safety of OSA surgeries under general anesthesia. A retrospective analysis was conducted on the clinical and follow-up data of four pediatric cases that experienced respiratory arrest after general anesthesia for OSA at Shenzhen Hospital of Southern Medical University from March 2020 to March 2022. All four children exhibited varying degrees of decreased blood oxygen saturation, cyanosis, and loss of consciousness after OSA surgery under general anesthesia, with one case experiencing respiratory and cardiac arrest.

Congenital vitamin D deficiency: presenting with feeding difficulty in early infancy: a case report.

Background: Vitamin D deficiency remains a significant public health concern, particularly among exclusively breastfed infants. Infants born to mothers with vitamin D deficiency, often influenced by cultural factors affecting diet, lifestyle, and clothing, are at increased risk of developing early and potentially fatal complications of hypocalcemic vitamin D deficiency. While seizures and tetany are well-recognized manifestations of hypocalcemia in infants, less common symptoms, such as feeding difficulties and recurrent apnea, are rarely documented.

[One case of successful treatment for acute poisoning of the pesticideAcetamiprid and pyridaben].

Acetamiprid and pyridaben is a compound pesticide, Due to its characteristics of high efficiency, and environmental safety, it has been widely used in agriculture in recent years.This article reports a case of poisoning from a mixture of pymetrozine and propargite pesticides. The patient experienced severe symptoms of poisoning such as vomiting, shortness of breath, abdominal discomfort, generalized cyanosis, bradycardia, and shock after self-administering the pesticide.

Digital Cyanosis as a Manifestation of Upper Limb Atherosclerosis: A Case Report.

In this case report, we present an atypical case of atherosclerosis manifesting as subclavian artery atherosclerosis in a 57-year-old male patient with a history of chronic smoking and dyslipidemia. The patient developed progressive cyanosis and vascular compromise in the fingers, characteristic symptoms of subclavian artery obstruction. The diagnosis was confirmed through Doppler ultrasound and computed tomography angiography, which revealed a 72% stenosis of the left subclavian artery.

Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries: The Natural History and Late Presentation.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries represents a heterogenous and complex congenital heart defect. Treatment pathways vary widely depending on center expertise. The recommended approach in infancy is to achieve uniform pulmonary artery and capillary bed development, and a biventricular repair when feasible.

A Case Report of Acute-on-Chronic Methemoglobinemia.

Introduction: Methemoglobinemia is a rare hematologic disorder of hemoglobin, in which iron contained within the heme moiety becomes oxidized from ferrous iron to ferric iron at a concentration greater than 1% in the blood. This biochemical change reduces binding affinity for oxygen, leading to impaired oxygen deposition in tissues and subsequent hypoxia and hypoxemia. The etiology of methemoglobinemia is often acquired from exposure to oxidizing agents, commonly antibiotics such as dapsone or local anesthetics such as benzocaine.

Unusual presentation of anomalous origin of the right pulmonary artery from the ascending aorta: case report.

Background: Anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta is a rare congenital anomaly, representing approximately 0.12% of all congenital heart defects. Early diagnosis and timely intervention are essential to prevent severe complications such as heart failure and pulmonary vascular disease.

[From persistent "hypoxemia" to the diagnosis of an hemoglobinopathy].

Low oxygen (O2) affinity hemoglobin (Hb) variants are a group of structural hemoglobinopathies, caused in most cases by point mutations in beta or alpha globin genes. The clinical presentation is widely variable, from asymptomatic patients to those presenting cyanosis and/or low O 2 saturation without signs of chronic hypoxia. Accurate identification of these patients is essential to avoid invasive cardiorespiratory procedures that could be unnecessary.

Propranolol palliation after right ventricular outflow tract stenting reduces the reintervention rate until complete repair of Fallot tetralogy and variants.

Introduction: Stenting of the right ventricular outflow tract (RVOT) is a reasonable palliation in symptomatic infants with tetralogy of Fallot (ToF) and variants. However, this procedure needs reintervention until corrective surgery.

Aim: To compare RVOT stenting followed with or without propranolol medication until complete repair of ToF and variants.

Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages.

Objective: Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months.

Chiari Network With Associated Cyanosis in a 3-Year-Old Girl.

We reported a 3-year-old girl with cyanosis. The patient was diagnosed with an obstructive Chiari network, which is an unrecognized cause of cyanosis. Echocardiography was used to diagnose this disease and provide additional information on flow and pressure gradients.

Raynaud Syndrome Associated with Medication for Attention-Deficit/Hyperactivity Disorder: A Systematic Review.

Background: Raynaud syndrome (RS) is a peripheral vasculopathy characterised be impaired acral perfusion typically manifesting as skin discolouration with pallor, cyanosis and/or erythema, and increased sensitivity to cold. RS may be primary or secondary to systemic disease, lifestyle and environmental factors or medication. RS has been reported with medication to treat ADHD, but we found no recent comprehensive overview of the literature.

Non-bacterial thrombotic endocarditis in a patient with high-grade serous ovarian carcinoma: diagnostic challenges and management insights.

Non-bacterial thrombotic endocarditis (NBTE) is characterised by sterile vegetations on heart valves and often emerges in hypercoagulable states like malignancy. It is frequently underdiagnosed and only comes to light during postmortem examination. Early diagnosis and treatment with anticoagulation can help lower mortality.

A fatal case of accidental asphyxia following nitrous oxide inhalation and alcohol consumption.

Nitrous oxide (NO) abuse is becoming increasingly popular worldwide. Moreover, the use of NO combined with other substances, such as alcohol, is also common. Accidental deaths associated with NO abuse are rare in forensic practice, with most fatal cases involving continuous inhalation equipment or exposure in a confined space.

Caval vein obstruction resulting in right to left shunt and desaturation post-endocardial pacing implantation in a 3-year old: a case report.

Background: Superior caval vein obstruction is a rare complication of endocardial pacing lead implantation that can result in a right to left shunt.

Case Summary: A 3-year-old child with type 2 Brugada syndrome presented with mild cyanosis post-endocardial pacing implantation due to evolutionary right superior caval vein obstruction. This obstruction resulted in a right to left shunt across a previously unrecognized patent levo-atrial cardinal vein associated with partial anomalous pulmonary venous drainage.

Severe Neonatal Asphyxia Associated with Ureaplasma urealyticum Infection: A Case Report.

BACKGROUND Ureaplasma urealyticum (UU) is a common microorganism that has been associated with a variety of obstetric and neonatal complications, such as infertility, stillbirth, histologic chorioamnionitis, neonatal sepsis, respiratory infections, and central nervous system infections. However, it is rare for it to cause severe neonatal asphyxia. This rarity is the focus of our case report, which aims to highlight the potential severity of UU infections in newborns.

Case report: Multisystemic smooth muscle dysfunction syndrome: a rare genetic cause of infantile interstitial lung disease.

Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an autosomal dominant disorder caused by mutations in the gene, resulting in variable clinical manifestation and multi-organ dysfunction. Interstitial lung disease (ILD) is a rare phenotype of this condition. We describe a rare infant case of an 8-month-old boy who presented with progressively worsening dyspnea, along with intermittent episodes of respiratory distress and cyanosis since birth.

Idiopathic pulmonary arteriovenous malformation: a rarity in clinical practice.

Pulmonary arteriovenous malformations (PAVM) are characterized by abnormal pulmonary vessels forming arteriovenous shunts that compromise oxygenation of the blood, causing hypoxemia, and predispose to infections and cerebral ischemia. The patient in this case was a 38-year-old male who presented with tachypnea and dyspnea, cyanosis of extremities, and significant digital clubbing. The patient had structural epilepsy secondary to neurosurgery for a cerebral abscess during childhood.

[Severe malnutrition during pregnancy complicated with acute pyelonephritis causing sepsis, refractory septic shock and multiple organ failure: A case report].

This study reports the diagnosis and treatment of a 26-year-old pregnant woman with severe malnutrition combined with acute pyelonephritis causing sepsis, refractory septic shock and multiple organ failure. A female patient, 26 years old, was admitted to hospital mainly due to "menelipsis for more than 19 weeks, nausea and vomiting for 20 days, fever with fatigue for 3 days". At the end of 19 weeks of intrauterine pregnancy, the patient presented with fever accompanied by urinary tract irritation.

Three cases of hemoglobin M disease in a family lineage: Case report and literature review.

Rationale: This study presents a case of hemoglobin M disease (HMD), a rare inherited disorder characterized by persistent cyanosis and hypoxemia, observed across 3 generations within a single family. The diagnosis of HMD poses significant challenges, particularly in asymptomatic individuals, due to its rarity and the subtlety of its symptoms. Notably, there is a scarcity of reports on methemoglobinemia in pediatric populations, which further complicates early detection and intervention.

Digit Amputation Secondary to COVID-19-Induced Raynaud's Phenomenon.

Raynaud's phenomenon (RP) is characterized by episodic vasospasm of the small blood vessels, primarily affecting the fingers and toes. Management includes lifestyle modifications, pharmacological treatments, and in severe cases, surgical interventions. Here we report a case of an 80-year-old male patient with a history of hypertension, dyslipidemia, obesity, and atrial fibrillation who presented to the emergency department with edema, cyanosis, and intense pain in the fingers of both hands following a mild COVID-19 infection (no dyspnea or hypoxemia).

Application-based remote interstage home monitoring for infants with shunt- or duct-dependent pulmonary perfusion.

Objective: Interstage home monitoring (IHM) programs are considered standard of care after Norwood palliation and have led to substantial improvements in clinical outcomes. This study aims to evaluate an application-based remote IHM program for infants with shunt- or duct-dependent pulmonary circulation. The primary goals were to discharge infants from the hospital while minimizing mortality, optimizing somatic growth, and enhancing caregivers' confidence in the clinical management at home.

Heterotaxy syndrome, dextrocardia, ureteropelvic obstruction, endometriosis, and pulmonary hypertension in an adult with congenital heart defects: a case report.

Background: Heterotaxia is characterized by an abnormal positioning of the thoracic and/or abdominal organs, resulting in various physiological and hemodynamic implications. Congenital heart disease involves structural irregularities in the heart or major vessels within the chest, leading to functional challenges.

Case Presentation: We present a 26-year-old Arab female patient with a complex medical history involving heterotaxy, dextrocardia, congenital heart disease, and ureteropelvic junction obstruction diagnosed in her first year of life, followed by the identification of endometriosis in her early twenties.

Diagnosis and management of residual atrial septal defect after surgical failure: A case series.

Residual defect after surgical closure of atrial septal defect is extremely uncommon. This communication reports four cases encountered in a tertiary care center during the last three decades. Clinical diagnosis was challenging, and the diverse presentations included acute ischemic stroke, cyanosis, and right ventricular volume overload.