Novel transcatheter intervention for iatrogenic cyanosis due to surgical rerouting of inferior vena cava to the left atrium.

Improper identification of the atrial septal defect margins during surgery and inadvertent suturing of the surgical patch to the Eustachian valve of the inferior vena cava (IVC) results in the diversion of inferior venacaval blood to the left atrium causing cyanosis. This complication has been dealt so far with surgery. We report the planning and implementation of a novel transcatheter rediversion of the IVC to the right atrium using a covered stent.

Pulmonary reperfusion injury in post-palliative intervention of oligaemic cyanotic CHD: a new catastrophic consequence or just revisiting the same old story?

Pulmonary reperfusion injury is a well-recognised clinical entity in the setting pulmonary artery angioplasty for pulmonary artery stenosis or chronic thromboembolic disease, but not much is known about this complication in post-palliative intervention of oligaemic cyanotic CHD. The pathophysiology of pulmonary reperfusion injury in this population consists of both ischaemic and reperfusion injury, mainly resulting in oxidative stress from reactive oxygen species generation, followed by endothelial dysfunction, and cytokine storm that may induce multiple organ dysfunction. Other mechanisms of pulmonary reperfusion injury are "no-reflow" phenomenon, overcirculation from high pressure in pulmonary artery, and increased left ventricular end-diastolic pressure. Chronic hypoxia in cyanotic CHD eventually depletes endogenous antioxidant and increased the risk of pulmonary reperfusion injury, thus becoming a concern for palliative interventions in the oligaemic subgroup. The incidence of pulmonary reperfusion injury varies depending on multifactors. Despite its inconsistence occurrence, pulmonary reperfusion injury does occur and may lead to morbidity and mortality in this population. The current management of pulmonary reperfusion injury is supportive therapy to prevent deterioration of lung injury. Therefore, a general consensus on pulmonary reperfusion injury is necessary for the diagnosis and management of this complication as well as further studies to establish the use of novel and potential therapies for pulmonary reperfusion injury.

Phlegmasia Cerulea Dolens in the Upper Extremity: A Case Report and Literature Review.

Phlegmasia cerulea dolens (PCD) is a rare condition characterized by deep venous thrombosis with gangrene. It can result in critically severe edema that affects the blood supply to the limbs. PCD generally occurs in the lower rather than upper extremity. We herein present a case report of upper extremity PCD and discuss thrombophilia secondary to low protein S activity as the main cause. Catheter-directed thrombolysis via the occluded end of the artery may be one of the best treatment methods for PCD.

High origin of the right coronary artery with partial anomalous pulmonary venous connection to the left superior caval vein in tetralogy of Fallot.

The prevalence of anomalous origin of coronary artery in tetralogy of Fallot has been reported to be around 4% to 6%. The association of high take-off of the right coronary artery from the distal part of the ascending aorta in tetralogy of Fallot in the presence of a partial anomalous pulmonary venous connection (PAPVC) to the left superior caval vein draining into the left atrium is not known to the best of our knowledge. We herein describe such a case when the anomalous right coronary artery and the PAPVC were detected incidentally during intracardiac repair; signifying the importance of a thorough assessment of the anatomy before surgery.

Cyanotic Congenital Cardiac Disease and Scoliosis: Pre-Operative Assessment, Surgical Treatment, and Outcomes.

OBJECTIVE: Congenital heart disease (CHD) is associated with the development of scoliosis. Improvements in cardiac care have extended survival of children with cyanotic CHD which possess a need for correction of scoliosis. There is limited information on spinal care for these patients. We present 3 patients with CHD who underwent surgical correction of scoliosis. MATERIALS AND METHODS: We reviewed demographic and clinical data on patients with cyanotic CHD. RESULTS: Patient 1 underwent posterior spinal fusion T3-L3 at the age of 16 years. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used but he lost 3,000 mL of blood. The operative time was 370 min and most of the blood loss occurred in the second half of the procedure. Patient 2 underwent posterior spinal fusion T5-T12 when aged 14 years. She had transposition of the great vessels corrected over multiple surgeries. Hypotensive anaesthesia was used, she had blood loss of 300 mL, and the surgical time was 282 min. Patient 3 underwent posterior spinal fusion extending from T5-T12 when he was 17 years old. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used, he had blood loss of 1,021 mL, and a surgical time of 342 min. CONCLUSION: Scoliosis surgery in patients with complex cardiac disease may be indicated to treat progressive deformities which produce severe symptoms. A multidisciplinary approach including a spinal surgeon as well as a cardiologist, haematologist, respiratory paediatrician, and spinal anaesthetist can evaluate the general medical condition and weigh the benefits and risks of surgery. Deformity correction can be performed using a meticulous technique and has produced a series of satisfactory outcomes.

Early or late fresh frozen plasma administration in newborns and small infants undergoing cardiac surgery: the APPEAR randomized trial.

BACKGROUND: In newborns and small infants undergoing cardiac surgery with cardiopulmonary bypass (CPB) and blood priming, it is unclear whether there is reduced blood loss if fresh frozen plasma (FFP) is added to the CPB priming volume. This single-centre, randomized trial tested the hypothesis that the administration of FFP after CPB (late FFP group) is superior to FFP priming (early FFP group) in terms of postoperative bleeding and overall red blood cell (RBC) transfusion. METHODS: Seventy-three infants weighing <10 kg were randomly allocated to receive FFP to supplement RBCs in the CPB priming solution ( n =36) or immediately after CPB ( n =37). The primary endpoint was a difference in postoperative blood loss; secondary endpoints included the amount of RBCs and FFP transfused through the first 48 postoperative hours. RESULTS: All patients were included in the analysis. Patients in the late FFP arm had greater postoperative mean blood loss than patients in the early FFP arm [33.1 ( sd 20.6) vs 24.1 (12.9) ml kg -1 ; P =0.028], but no differences in transfusions were found. The subgroup of cyanotic heart disease patients had comparable results, but with greater use of RBCs in the late FFP group. CONCLUSIONS: In infants undergoing cardiac surgery, FFP in the priming solution appears slightly superior to late administration in terms of postoperative bleeding. CLINICAL TRIAL REGISTRATION: www.ClinicalTrials.gov , NCT02738190.

Liver transplantation after stage II palliation for hypoplastic left heart syndrome.

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.

Two isolated giant gastric duplication cysts in thorax in a newborn.

We report a case of a newborn with severe respiratory distress since birth with two giant intrathoracic and separate eneteric duplication cysts in right hemithorax. On day 19, the intrathoracic cysts were removed, and the baby was discharged on his 22nd day of life. Histologic findings confirmed the diagnosis of a gastric duplication cyst. This report is the first case of two isolated, separated and giant right intrathoracic gastric duplication cysts in literature. The diagnostic values of radiological evaluation and surgical and pathological management for precise diagnosis are discussed.

Erythromelalgia in a patient with feet erythema and cyanosis.

Erythromelalgia is a rare disease characterised by a triad of a clinical syndrome of redness, warmth and painful extremities. We present the case of a male adolescent with no prior medical history who presents to our family medicine clinic with a 3-month history of bilateral feet erythema followed by episodes of cyanosis in bilateral toes. Given his history, the findings on clinical examination, and the lack of any pathology on the diagnostic testing, the patient is diagnosed with erythromelalgia. He is then counselled on both pharmacological and non-pharmacological treatments for his condition and is discharged on non-pharmacological treatment options such as leg elevation, cooling with a fan and limiting exposure to heat. The patient is also advised to perform an annual complete blood count given the association of erythromelalgia with myeloproliferative disorders.

[Heart failure in young children; a serious cause of feeding problems]. / Hartfalen bij jonge kinderen.

Feeding problems in children are usually harmless and common, but can rarely exist as a result of pathology. Heart failure is one of them and has to be recognized early because of its many consequences. We present a 15-week-old female infant who was seen at the outpatient clinic. She had already been evaluated several times by a youth doctor and general practitioner because of feeding problems and transpiration, for which several nutritional interventions had already been carried out. With no effect of nutritional interventions she was referred to a pediatrician. Physical examination showed clear signs of heart failure and echocardiogram showed a severe dilation of the left ventricle, with poor contractility of the lateral wall and papillary muscle, with end stage heart failure due to an Anomalous Left Coronary Artery from the Pulmonary Artery (ALPACA). In this article we discuss the clinical presentation of heart failure: stagnant growth, exercise intolerance, tachypnea and sometimes hepatomegaly, edema, murmurs or cyanosis. In addition, when a patient does not respond to initial therapy, we recommend to reconsider differential diagnosis and/or refer to a pediatrician.

Ventricular fibrillation in loop recorder memories in a patient with early repolarization syndrome.

We report the first documentation of spontaneous ventricular fibrillation by a loop recorder in a patient with an ECG pattern of early repolarization (ER) in the inferior leads and presenting with syncope.

Bicameral repair of right pulmonary artery to left atrial communication.

A right pulmonary artery to left atrial communication is a very rare vascular congenital anomaly. Patients most commonly present in the neonatal period with congestive cardiac failure or at a later stage with central cyanosis and its complications. Various diagnostic modalities are available but angiography is the most important decision-making tool for the management of this lesion. We present an unusual case of right pulmonary artery to left atrial communication in a 14-year-old patient, who underwent successful surgical repair through a bicameral approach.

Cyanosis in a 9-month-old child after repair of total anomalous pulmonary venous return.

A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.

Impact of hypoxemia and re-interventions on clinical outcomes after bidirectional cavopulmonary shunt.

OBJECTIVES: We sought to investigate the impact of early postoperative low arterial oxygen saturation on mortality and morbidity after bidirectional cavopulmonary shunt (BCPS). METHODS: The medical records of all patients who underwent BCPS between 2013 and 2018 were reviewed. RESULTS: A total of 164 patients were included in this study. Forty-seven patients underwent reintervention during hospital stay at median 7 days after BCPS. Before reintervention, 30 patients were intubated or had SpO2 of <75%. All re-interventions for Glenn pathway obstruction and 4 out of 5 venovenous coil embolization resulted in hospital discharge, while high mortality was observed after other re-interventions (atrioventricular valve surgery, thrombolysis, systemic ventricular outflow obstruction relief, extracorporeal membrane oxygenation implantation and diaphragmatic plication). Additional aortopulmonary shunt with pulmonary artery discontinuation was performed in 8 patients who showed severe cyanosis with median SpO2 of 59% under maximal ventilation support. In the univariable Cox regression analysis, the associated factors for mortality before total cavopulmonary connection were reduced ventricular function [hazard ratio (HR) 6.89, 95% confidence interval (CI) 1.76-26.9, P-value 0.006], greater than moderate atrioventricular valve regurgitation (HR 5.89, 95% CI 1.70-20.4, P-value 0.005), SpO2 1 h after extubation (HR 0.87, 95% CI 0.80-0.96, P-value 0.004) and mean pulmonary artery pressure 1 h after extubation (HR 1.14, 95% CI 1.02-1.26, P-value 0.016). CONCLUSIONS: After BCPS, unacceptable cyanosis persisted with various aetiologies. Low arterial oxygen saturation within 1 h after extubation is significantly associated with high mortality after BCPS.

Case report: Pulmonary artery thrombosis in cyanotic congenital heart disease.

Cyanotic congenital cardiac anomalies increase the risk of thrombosis. In pediatric congenital heart disease, the diagnosis of pulmonary artery thrombosis is rare. We reported an 11-year-old male child who developed progressive shortness of breath over six months. He was subsequently referred to the National Institute of Cardiovascular Diseases, Karachi, Pakistan. The child was diagnosed as a case of double outlet left ventricle, levo-transposition of the great arteries, pulmonary stenosis, ventricular septal defect and thrombus in the main pulmonary artery with extension into the branch pulmonary arteries. He underwent thromboendarterectomy with a Glenn shunt and made an uneventful postoperative recovery.

Respiratory arrest due to airway obstruction following endoscopic removal of Trichobezoar.

A trichobezoar is a ball of swallowed hair that collects in the stomach and fails to pass through the intestines. Upper gastrointestinal endoscopy is usually useful for diagnosis and retrieval of a portion of the gastric Trichobezoars but can have complications. Upper airway obstruction may occur during removal of bezoar. This complication may be life threatening. We report the case of a 17-year-old girl who presented with severe anaemia and hypoproteinaemia. During diagnostic upper GI endoscopy, a large piece of hairball compressed the upper airway, leading to cyanosis, respiratory arrest and cardiovascular collapse. The patient was immediately intubated and transferred to the operation room for emergency esophagoscopy. In the operation room, after haemodynamic stabilization the otolaryngologist removed the large piece by esophagoscope. Then the patient was transferred to ICU ward with spontaneous ventilation. The day after, she became stable and conscious.

Evaluation and management of apparent life-threatening events in infants.

Apparent life-threatening events (ALTEs) are frightening to caregivers and clinicians alike. This article provides a comprehensive review of the causes, management, and consequences of ALTEs. The information provided was collected from an extensive literature review using the search terms ALTE, sudden infant death syndrome, and apnea. There is a wide array of contributing factors to ALTE syndrome and sequalae for both infants and caregivers of infants experiencing an ALTE.

Congenital bilateral choanal atresia.

Congenital choanal atresia (CCA) is the developmental failure of the nasal cavity to communicate with nasopharynx. Surgical repair is recommended in the first weeks of life in bilateral cases because this is a life-threatening situation in newborns. This is a case report of a full-term, healthy newborn baby presenting with intermittent attacks of cyanosis and respiratory distress soon after birth. On examination, alternating cyanosis and normal colour was observed in the infant. The insertion of nasal catheters in both the nares revealed the diagnosis of bilateral CCA. For this rare condition, conservative management was followed with elective transnasal endoscopic repair to relieve the life threatening nasal obstruction at the tenth day of life.