Cyanotic Spells Research Articles

Outcomes of surgical treatment of tracheobronchomalacia in children.

Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptomsas well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student'st-test for continuous data. Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Palliative Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot with Severe Cyanosis: Experience from a Single Center in Southern India

Background: This study conducted at an Indian tertiary care hospital aimed to characterize the clinical profile of tetralogy of Fallot (ToF) patients undergoing right ventricular outflow tract (RVOT) stenting and assess their immediate postprocedural outcomes until discharge. Methods: In this retrospective analysis, the clinical and procedural outcomes were analyzed for 20 patients who underwent palliative stenting in the RVOT for severe cyanosis associated with ToF during 2021–2023. Results: The median age for the patients was 2.3 years (range 3 months–21 years), two being adults (>18 years). Fourteen (70%) patients underwent elective RVOT stent and 30% underwent emergency stenting. The most common indication of RVOT stenting was hypoplastic branch pulmonary arteries (PAs) (n = 11, 78.6%) and cyanotic spell (n = 4, 66.7%) for elective and emergency surgeries, respectively. The average improvement in SpO2 was 27.2% (95% confidence interval 23.4–31.0, P < 0.001). Six (30%) patients had complications including reperfusion injury of lungs (n = 2, 33.4%) sepsis (n = 1, 16.7%), stent embolization (n = 1, 16.7%), re-stenting (n = 1, 16.7%), transient complete heart block (n = 1, 16.7%) during the procedure, and. One child went through RVOT stent postoperative due to severe residual stenosis and child developed severe tricuspid regurgitation and succumbed to death due to culture positive sepsis with multi-organ dysfunction. Rest (n = 19, 95%) were discharged with an average 6.7 days of hospital stay. Conclusion: ROVT stenting is a safe and effective palliative management in ToF enhancing oxygen saturation prior to definitive surgical repair.

Echocardiographic Study of Ventricular Septal Defect in 1- to 12-Year-Old Children Visiting a Tertiary Care Center in Patna, India.

Background Symptoms of serious heart problems present at birthoften appear during the first few days, weeks, or months of a child's life. Shunt formation between the left and right ventricle is a crucial component of the pathophysiology of ventral septal defects. Objectives We aim to determine the most frequent types of ventricular septal defect (VSD) by echocardiography and whether there is any gender variation in the type of ventricular septal defect. Material and methods A total of 100 children who were clinically suspected of or diagnosed with VSD at the age of 1-12 years were enrolled in this study. The septum of the atrioventricular (AV) canal, the muscular septum, and the parietal band of the distal conal septum were evaluated by color Doppler. Ventricular septal defect (VSD) size and kind are similarly impacted by the 2D echo mode. The size and site of the VSD, associated congenital anomaly, and significant morphological changes in ventricular cavities, gender discrimination, and relation-specific types of ventricular septal defect were observed. Results A total of 100 VSD children presentedwith clinical symptoms of fast breathing, retraction of the chest, cough, cyanosis, fever, difficulty during feeding, cyanotic spell, chest pain, and edema at 65%, 62%, 54%, 52%, 54%, 29%, 9%, 11%, and 4%, respectively. Conclusion Early diagnosis is essential for effective medical care of diseases such as infective endocarditis (IE), which is present in some cases of VSD, and the avoidance of persistent pulmonary veno-occlusive disease (PVOD).

Heterotaxy Syndrome In A Middle-Aged Pakistani Male

Background: Heterotaxy syndrome (or situs ambiguous) is an extremely rare disorder in which the viscera are arranged in an abnormally asymmetrical pattern around the midline along with cardiac and spleen abnormalities. The management of this disorder depends upon the extent and variability of the organ involvement and a multi-disciplinary approach is often required.
 Case presentation: A middle-aged male presented in pulmonology OPD with with complaint of hemoptysis and shortness of breath for 3 weeks. He had a history of cyanotic spells since childhood. His HRCT chest revealed dextrocardia, generalized mild centrilobular emphysema of the lungs with fibrotic bands and mild cylindrical bronchiectasis and his echocardiography showed dextrocardia along with ventricular septal defect and pulmonary hypertension. He was found to have abnormally positioned abdominal viscera along with multiple spleens and he was diagnosed as a case of heterotaxy syndrome with left isomerism. His respiratory symptoms were treated conservatively and the patient was referred to the cardiology unit for management of cardiac defects.
 Conclusion: This manuscript describes a case of heterotaxy syndrome which is a rare disorder with significant mortality and morbidity. The patient may present with vague symptoms. Early involvement of all the relevant specialities might help in a prompt diagnosis and timely management, which may improve the disease outcome.

Outcome of Iron Deficiency Anemia in patients presenting with Breath Holding Spellsat Lady Reading Hospital Peshawar

Aim: To ascertain the course of iron deficiency anemia in patients who presented to OPD and wards with breath-holding episodes. Methodology: From June 19 through December 18, 2019, the pediatrics department of Lady Reading Hospital in Peshawar conducted this study with ethical committee approval. The study included all kids aged 1 to 5 years, both genders, and those who had Cyanotic Breath spell lasting two or more episodes. Children with Cyanotic congenital heart disease, a history of febrile convulsions or epilepsy, anticonvulsive therapy currently being used, delayed children, and malnutrition in the past were not included in the study. Results: In our 161 patients, the average age was 2.671.811, the average weight was 12.913.33kg, and the average length of illness was 6.834.24 months. Males made up 94(58.4%) and females 67 (41.6%) of the patient population, while PICA children made up 72(44.7%). Iron deficiency anemia was present in 87(54%) of the patient population, but not in 74(46%), who had it. PICA children and children who were breastfed exclusively had a significantly higher correlation with iron deficient anemia. Conclusion: It is highly typical for patients who experience breath holding spells to have iron deficient anemia. These patients should receive an accurate diagnosis of iron deficiency anemia and treatment to soothe the parents' concerns and limit the frequency of unneeded hospital visits. Key words: iron deficiency anemia, breath holding spells, PICA child

Large cameral coronary artery fistula in a 5 months old infant with unusual presentation and fatal outcome: -case report

BackgroundCongenital coronary fistulas (CAFs) are uncommon abnormalities communicating the coronary arteries with the cardiac chambers or portion of the systemic or pulmonary circulation. Over 90% of the cases drain into the right side of the heart with only 3% terminating in the left ventricle. Infants with a large CAFs may develop congestive heart failure.Case presentationA 5 months old female infant presented with labored breathing and worsening of bluish discoloration of the lips and extremities following a prolonged cry. She had a history of breastfeeding difficulty and noticeable bluish discoloration of the lips and extremities since birth. The infant was wasted and had a fast heart rate, bluish lips, and nail beds with clubbing of fingers and toes. A cardiac murmur was noted during her medical checkup. Chest x-ray showed cardiomegaly. Echocardiography and CT angiography showed large Cameral CAF involving the left main and left anterior descending artery draining into the left ventricle. The tricuspid valve was dysplastic, there was secundum ASD, and VSD with a right to left shunt. The patient developed episode of cyanotic spells after crying excessively following a CT angiographic procedure which culminated in respiratory arrest and her demise. She was managed as a case of hypoxic spells in the ICU before her death.ConclusionThis report unveiled unfamiliar case of Cameral coronary artery fistula with left-to-left shunting, cyanosis, and dysplastic tricuspid valve.

Consensus recommendations on the evaluation and treatment of congenital laryngeal clefts

Congenital laryngeal cleft is a rare airway malformation, mainly manifested as choking, feeding difficulties, which affects the growth and development of children. Patients with a severe laryngeal cleft may have recurrent aspiration, leading to cyanotic spells, or even death. Advances in development of endoscopic techniques have made early diagnosis possible. Depending on the degree of cleft, management may involve a variety of approaches ranging from medical management alone to open repair. Therefore, it is important for pediatric ENT doctors to diagnose and evaluate in clinical practice. This consensus statement, developed by the Pediatric otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance on diagnosis and management of laryngeal cleft, based on symptomatology, physical examinations, and laboratory tests.

Pattern of Congenital Heart Diseases among Less than 5 Years Old Children of Bangladesh-An Observational Study from a Pediatric Cardiac Center

Introduction: Congenital Heart Disease (CHD) is the most common congenital problems. CHD is about 25% of all congenital malformations and is the most common type of heart disease among children. It may present in different ages from birth to adolescence. Worldwide, CHD is relatively common with a prevalence ranging from 1.0-6.6 per 1000 live births. Aim of the Study: The aim of this study was to find out the pattern of congenital heart diseases among less than 5 years old Children of a pediatric cardiac center. Methods: This was a retrospective observational study and conducted in the Department of pediatric cardiology of Combined Military Hospital Dhaka, Bangladesh during the period from July, 2018 to July, 2020. A total of 1000 patients from outdoor and indoor patient registry who were diagnosed and treated as a case of congenital heart disease within the mentioned period were included in the study. Result: Majority of our patients was male (58%) compared to female (42%). Among all patients, 29.80% had VSD, followed by 20.4% & 14.9% had ASD & TOF respectively and 72% & 28% of our patients had Acyanotic & cyanotic CHD respectively.VSD & TOF was the most common in patients with acyanotic & cyanotic CHD respectively. Recurrent RTI, pneumonia, heart failure, infective endocarditis, and Cyanotic spell are common complications of congenital heart disease. Down syndrome and congenital rubella syndrome are the main syndromic association in congenital heart diseases. The majority (97%) of our patients improved, and 3% died. Conclusion: Our study focuses on the congenital heart diseases of children of Bangladesh. Among the acyanotic congenital heart disease, VSD is the maximum & among the cyanotic congenital heart disease TOF is the highest in number. With early diagnosis, treatment and regular follow up, mortality rate is very low and the prognosis is excellent.

A uniform strategy of primary repair of tetralogy of Fallot: Transventricular approach results in low reoperation rate in the first decade

ObjectivesTo review outcomes after a uniform strategy of transventricular repair of tetralogy of Fallot. MethodsA total of 244 consecutive patients underwent transventricular primary repair of tetralogy of Fallot from 2004 to 2019. Median age at operation was 71 days; 57 (23%) patients were premature; 57 (23%) patients had low birth weight (<2.5 kg), and 40 (16%) had genetic syndromes. The diameter of pulmonary valve annulus, right pulmonary artery (PA), and left PA were 6.0 ± 1.8 mm (z score, −1.7 ± 1.3), 4.3 ± 1.4 mm (z score, −0.9 ± 1.2) and 4.1 ± 1.5 mm (z score, −0.5 ± 1.3). ResultsThree (1.2%) operative deaths were recorded. Ninety patients (37%) underwent transannular patching. Postoperative echocardiographic peak right ventricular outflow tract gradient decreased from 72 ± 27 mm Hg to 21 ± 16 mm Hg. Median intensive care unit and hospital stay were 3 and 7 days. The survival rate at 10 years was 94.6% ± 1.8%. Reintervention was required 86 times (55 catheter interventions) in 56 patients following tetralogy of Fallot repair. The freedom from all-cause reintervention rate at 10 years was 70.5% ± 3.6%. Cyanotic spells (hazard ratio, 2.14; 95% CI, 1.22-3.90; P < .01) and smaller pulmonary valve annulus z score (hazard ratio, 1.26; 95% CI, 1.01-1.59; P = .04) were associated with increasing risk of all reinterventions. Freedom from redo surgery for right ventricular outflow tract obstruction and right ventricular dilatation at 10 years were, respectively, 85.0% ± 3.1% and 98.7% ± 0.9%. Freedom from valve implantation was 96.7% ± 1.5% at 10 years. ConclusionsA uniform strategy of primary repair of tetralogy of Fallot through a transventricular approach resulted in low reoperation rate in the first decade. The need of pulmonary valve implantation was limited to <4% at 10 years.

Consensus recommendations on the evaluation and treatment of laryngotracheal anomalies in infants and young children

Infants with laryngotracheal anomalies are clinically manifested as stridor or noisy breathing, choking, hoarseness, feeding difficulties, and cyanotic spells, followed by developmental and growth retardation and other health issues; in severe cases, patients may present with severe dyspnea, which is associated with high mortality. A timely diagnosis as well as appropriate strategy for laryngotracheal anomalies is still challenging for pediatric otolaryngologists. This consensus statement, evolved from expert opinion by the members of the Pediatric Otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance for otolaryngologists who manage infants and young children with laryngotracheal anomalies in evaluation and treatment based on symptomatology, physical and laboratory examinations.

Antioxidant levels in children with cyanotic breath-holding spells

Background Breath-holding spells (BHSs) are paroxysmal nonepileptic attacks that occur in some infants and toddlers. Their pathophysiology and etiology are still incompletely well defined. Aim To measure the serum levels of some antioxidant markers in children with cyanotic BHSs. Patients and methods This cross-sectional study included 50 children with cyanotic breath-holding attacks and 50 healthy control children, recruited from Pediatric Neurology Clinic and General Pediatric Clinic, Pediatric Department, Tanta University Hospital. Serum total antioxidant capacity and catalase activity were measured by the spectrophotometric method. Results Total antioxidant capacity and catalase activity levels were significantly lower in children with cyanotic BHS than in normal control children (P&lt;0.05). The number of breath-holding attacks was significantly negatively correlated with total antioxidant capacity (r=-0.77, P=0.03) and catalase activity levels (r=-0.8, P=0.04). Conclusion Children with cyanotic BHS have significantly lower levels of total antioxidant capacity and catalase activity.

Acute Esophageal Necrosis Secondary to Cyanotic Spells Associated with Unrepaired Tetralogy of Fallot.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease. Cyanotic spells occur more frequently after infancy in unrepaired cases. Acute esophageal necrosis (AEN) is a rare disease that causes circumferential mucosal necrosis in the distal esophagus. We report the case of a 26-year-old man who was admitted due to coffee-ground emesis, black stools, and decreased oxygen saturations. The patient had an unrepaired ToF and a congenital portosystemic venous shunt. An upper gastrointestinal endoscopy revealed AEN, which could be due to unstable hemodynamics of cyanotic spells. This is the first adult case presenting these 2 conditions occurring simultaneously.

Unravelling the Convoluted Story of Perioperative Care in Three-year-old Child with Tetralogy of Fallot Undergoing Repair Surgery

Tetralogy Of Fallot (TOF), one of the most prevalent cyanotic congenital heart diseases in children. Single step corrective surgery, early on in life, provides a fair expectation of favourable outcome in these individuals. However, complex, and skilled anaesthetic management is required by experienced team of healthcare providers. Preoperative surgical preparation, intraoperative key anaesthesia principles and postoperative care intensive care unit are all perioperative considerations in these patients. This article reports challenges faced in the perioperative anaesthesic management of a 3-year-old child having uncorrected TOF, who underwent Waterston shunt and later Intracardiac Repair (ICR). He presented with postoperative complication like cyanotic spell, gastrointenstinal bleeding, sepsis; but later recovered successfully.

Temper Tantrums and Breath-Holding Spells

Temper Tantrums and Breath-Holding Spells

Тетрада Фалло та гіпертрофічна кардіоміопатія. Нетипове поєднання

Tetrallogy of Fallot (ToF) is the most widespread congenital heart defect characterized by a wide anatomic spectrum and clinical manifestations, which depend on the degree of stenosis of the pulmonary artery, and can be associated with chromosomal abnormalities. Hypertrophic cardiomyopathy (HCM) is a rare genetic disorder that often occurs in the autosomal-dominant type and has a high risk of cardiac death and is associated with abnormalities in certain gene loci. Clinical case. We present this rare association in a 9-month-old girl, without previous history of heart defect, who was admitted to intensive care unit with a clinical presentation of severe hypoxic spell. A rare case of the combination of ToF and HCM has been reported in a 9-month-old child admitted to the reanimation department of the Lviv Regional Children’s Clinical Hospital «OKHMATDYT» for malignant and cyanotic attacks. The girl was hospitalized for reanimation in a jaundice-cyanotic crisis with a saturation rate of 44%. On examination, a pronounced sciatica, pallor and cyanosis of the lips were detected. Auscultatively, the heart tones were rhythmic, muffled, the heart rate - 202 beats per second, systolic murmur was detected on the left side of the chest 5/6 on the Levine scale. Electrocardiogram showed signs of systolic hypertension and hypertrophy of the right ventricle. 2D-echocardiographic examination revealed hypertrophy of the left and right ventricular walls and interventricular septum as well as signs of ToF. On the magnetic resonance imaging the diagnosis was confirmed. After the stabilization of the general condition the child was transported to the Center of Pediatric Cardiology and Cardiac Surgery in Kyiv for surgical treatment, where it was recommended to continue the medical treatment with beta-blockers. After 2 months, the 1-year-old child was operated on routinely - radical correction of ToF, the post-operative state was good. Conclusions. Association of ToF and HCM is extremely rare. ToF is often associated with chromosomal aberration, while hypertrophic cardiomyopathy associates with certain gene loci. Surgical treatment of ToF associated with HCM differs greatly from surgical treatment of usual ToF and physiology of both conditions have to be considered prior to the surgery, as combination of ToF and HCM is associated with high postoperative mortality, as the LVOT progresses and increased risk of development of ventricular arrhythmias and heart failure develops. The association between ToF and HCM is extremely rare. ToF is associated with chromosomal abnormalities, whereas HCM is associated with abnormalities in certain gene loci. The prognosis for patients with TF and HCM is associated with high postoperative mortality due to progression of obstruction of the left ventricular tract, development of ventricular arrhythmias and cardiovascular failure during the postoperative period. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Key words: Tetrallogy of Fallot, hypertrophic cardiomyopathy, cyanotic spell, echocardiography, children.

Paediatric anterior larynx: presentations, associations and a review of the literature.

The literature on the entity of the anterior larynx (AL) is restricted to little evidence on the difficulty encountered in exposing the larynx for intubation, perioperative morbidity and mortality, and scant reports on its prevalence in general adults and children. Here, we describe the prevalence of AL in a series of children presenting with aerodigestive symptoms and explore its association with functional abnormalities, congenital and structural anomalies or conditions. Tertiary paediatric centre. We conducted a retrospective case-control study. Using a prospectively collected surgical database, we identified patients diagnosed with AL (Grades IIb-III-IV Modified Cormack-Lehane scale) and sex and age-matched controls who had undergone full airway endoscopy for aerodigestive symptoms. We collected the demographics, presentations, associated diagnoses and investigations. We compared the proportion of patients with large airway abnormalities and dysmorphism and estimated the prevalence of AL. Over a 5.5-year period, 58 children with AL (28 females, mean age 0.38years) were matched with 58 controls (mean age 0.42years). Although both groups presented with permutations of stridor, respiratory failure, cyanotic spells, swallowing and feeding difficulties and SDB, AL presented much more with swallowing and feeding problems (33 vs. 20, p<.05). There were significantly more children with dysmorphism in the AL group (29 vs. 9, p<.05). The prevalence of AL was 4.9% (SE 0.0063). AL was detected in 4.9% of a sample of children who had undergone airway examination for aerodigestive symptoms. It is significantly associated with dysmorphism, and swallowing difficulties that warrant instrumental evaluation.

THE OUTCOME OF PALLIATIVE STENTING IN CYANOTIC CONGENITAL HEART DISEASES

Objective: To determine the outcome of palliative stenting in children with cyanotic congenital heart diseases. Methodology: From February 2018 to October 2020, the Department of Paediatric Cardiology at Lady Reading Hospital in Peshawar conducted a cross-sectional study on patients who underwent right ventricular outflow tract blocked Blalock Taussig shunt stenting or patent ductus arteriosus stenting for cyanotic congenital heart disease. The patients' information was gathered using a pre-formatted proforma. Pre- and post-procedure oxygen satu­rations were recorded for 48 hours. The favorable outcome was defined as an increase in oxygen saturation of at least 15%. Results: The mean age of the sample (n=49) was 20±29 months. The etiology was Pulmonary Atresia (PA) with Patent ductus arteriosus (PDA) in 23 patients, followed by blocked Blalock Taussig Shunt (BT-Shunt) in 9 patients. The number of patients who underwent PDA stenting was 32, 9 patients had stenting of blocked BT-Shunt and 8 patients had RVOT stenting. The mean pre-intervention oxygen saturation was 43% ±8.9% and the mean post-in­tervention oxygen saturation was 81% ±5.3%. The number of patients who died was 2, one patient for RVOT stenting died on the table due to cyanotic spell while other post-PDA stenting died because of stent thrombosis. Conclusion: RVOT, Patent Ductus arteriosus, and blocked Blalock Taussig shunt stenting was found effective in increasing oxygen saturation in children with cyanotic PDA.

Emergency Management of Cyanotic Spell in a Covid-19 Suspect Tetralogy of Fallot Child

Abstract The novel coronavirus (SARS-CoV-2) is suggested to increase the risk of morbidity and mortality in patients with underlying cardiac and pulmonary disorders. Reports of COVID-19-related multisystem inflammatory syndrome in children, past experiences with influenza virus and the respiratory syncytial virus, and British Congenital Cardiac Association recommendation on congenital heart disease warrant caution even in children. We present a case of a 2-year-old child with tetralogy of Fallot, who got admitted in the emergency with cyanotic spell and fever (suspected to be due to COVID-19), potentially compounding the challenge of managing cyanotic spell emergency.

Emergency Department Presentation and Outcome of Children With Cyanotic Congenital Heart Diseases.

ObjectiveThis study aimed to assess the outcome and see the spectrum of presenting symptoms of children with cyanotic congenital heart diseases (CHDs) admitted through the emergency department in Lady Reading Hospital (LRH) in Peshawar, Pakistan.Materials and methodsThis cross-sectional study included 104 children with cyanotic CHD admitted through the emergency department in the pediatric cardiology unit of LRH from February 2019 to January 2020. Diagnosis of cyanotic CHD was confirmed through two-dimensional echocardiography. Patients were treated according to standard protocols for their presenting symptoms. The data were analyzed using Statistical Package for the Social Sciences (SPSS), version 20.0 (IBM Corp., Armonk, NY) and frequencies were expressed as percentages.ResultsThe study included 65 male cases (62.5%) and 39 female cases (37.5%), and participant ages ranged from 10 days to 15 years (mean, 2.7 ± 3.4 years). Of 104 patients, 70 presented with cyanotic spells (84.5%), 53 with fever (10.7%), 28 with respiratory distress (26.5%), 11 with loose stools (10.5%), 10 with hemiplegia (9.6%), and eight with seizures (7.6%). Pneumonia was a precipitating factor in 13 patients (12.5%), infective endocarditis in 12 patients (11%), gastroenteritis in 11 (11%), brain abscess and cerebrovascular accidents in seven patients each (6% each), meningitis in six patients (6%), and tuberculous pericardial effusion in one patient (<1%). The primary CHD was tetralogy of Fallot (TOF) in 52 patients, tricuspid atresia in 14 patients, pulmonary atresia in 13 patients, double outlet right ventricle in 10 patients, transposition of great arteries (TGA) in seven patients, and total anomalous pulmonary venous return (TAPVR), truncus arteriosus, congenitally corrected TGA, and critical pulmonary stenosis in two patients each. Twenty-six patients received treatment of the acute problem and instructions for follow-up monitoring. Twelve patients died during their hospital stay, and three left against medical advice. Fifteen patients received patent ductus arteriosus stenting, and six received right ventricular outflow tract stenting. One patient received a Blalock-Taussig (BT) shunt stent, and one received a left pulmonary artery stent. Two patients received a balloon pulmonary valvotomy, and one received pericardial effusion drainage. Eleven patients received surgical correction of TOF, 11 received surgical correction for BT shunt, four received surgical correction for brain abscess drainage, and two received TAPVR repair.ConclusionTOF was the most common cyanotic heart disease in our study. Cyanotic spells or increased cyanosis was the most common presenting concern. Cyanotic CHDs present with variable extracardiac signs and symptoms in emergencies. Appropriate assessment, immediate management of the acute problem, and timely intervention or surgical management result in good outcomes.

Palliative Balloon Pulmonary Valvotomy in Tetralogy of Fallot: Is There a Role in 2021?

The current treatment practice for Tetralogy of Fallot (TOF) is to undertake complete surgical repair between 6–9 months of age with excellent immediate and long-term results. In patients with increased cyanosis or frequent cyanotic spells, younger age of complete repair as early as 3 months is usually acceptable. Although neonatal TOF repair is reported with good immediate survival from a few centres, post-operative morbidity is significant, and the hospital stay is prolonged. Hence, in neonates and small term and preterm infants with severe cyanosis, palliative procedures such as modified Blalock-Taussig (BT) shunt, ductal stenting, right ventricular outflow tract (RVOT) stenting and balloon pulmonary valvotomy (BPV) have been reported. With the development of low-profile stents, an increasing clinician experience and more predictable outcome with both duct and RVOT stenting, these procedures have gained popularity as the preferred palliations for TOF at most centres. We reviewed the literature to analyse whether BPV for TOF palliation, a technique first reported three decades ago, still has a role in the present era of paediatric cardiac care. In this review, we have concluded that BPV has very specific indications in patients with TOF, with predominantly valvar pulmonary stenosis, and may be a preferred option over other palliative strategies in hypoxemic preterm or low-birth weight infants.