Search

Ventricular septal defects (VSDs) are a prevalent congenital heart anomaly demanding safe and lasting interventions. This paper explores the application of Invengenx® bovine pericardial patch (Tisgenx, Irvine, California), a promising biomaterial, in VSD repair. We present two case studies: a seven-month-old infant and a three-year-old child undergoing VSD closure using autologous and bovine pericardial patches, respectively. Both patients tolerated the procedures well, experiencing no intra-operative complications and demonstrating excellent postoperative recovery. Echocardiography postoperatively showed no complications and improved clinical outcomes. Notably, the pericardial patches exhibited excellent integration and suture retention, highlighting their durability and compatibility with the growing heart. These cases establish the feasibility and effectiveness of the Invengenx® pericardial patch for VSD repair. The favorable outcomes in terms of safety and efficacy support the potential of this biomaterial as a valuable alternative in pediatric cardiac surgery, particularly for complex VSDs or patients with contraindications to synthetic patches. Further research is crucial to unlock the full potential of bovine pericardium as a durable and advantageous option for VSD repair in a broader range of pediatric patients., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Bhende et al.)

Congenital pulmonary airway malformations (CPAM) compose the major part of congenital lung malformations (CLM) and have traditionally been treated by pulmonary lobectomy. In terms of surgical strategy, lobectomy has conventionally been the preferred treatment for CPAM localized to a single lobe. More recently, alternative approaches including lung-sparing resections (LSR), such as wedge or non-anatomic resections and segmentectomy, have been suggested. In asymptomatic CPAM early surgical resection is often shown to reduce infection and malignancy development. We describe two patients who were diagnosed with CPAM when being evaluated for respiratory tract infection. Patient 1 (P1) was a two-month-old infant weighing 4 kg with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Patient 2 (P2) was a toddler aged one year, nine months weighing 9 kg. P1 underwent LSR for the CPAM diagnosed in the left upper lobe of the lung with conventional mechanical ventilation whilst right upper lobectomy was performed in P2 using one/single lung ventilation. In both cases, LSR and right upper lobectomy led to an uneventful postoperative recovery with no complications reported., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Bhende et al.)

One-stage total correction is known to be anatomically unsuitable for correcting tetralogy of fallot (TOF) in a certain proportion of children. Surgeons are thus faced with dilemmas regarding which preliminary operation for the anomaly to do first. Brock's primary postulation suggests that pulmonary trunk and annulus enlargement leading to the correction of the outflow obstruction will favor the subsequent total correction. In line with this, the current article presents two patients who were 6 months and 5 years old. The first patient underwent primary Brock's operation while the second patient had a blocked modified Blalock-Taussig's shunt (MBTS) done off-pump. Following the discontinuation of anti-platelet medications, the MBTS blocked and the patient was subsequently considered for secondary Brock's operation. The outcome of both procedures involved the patients' discharge with uneventful hospital stays and regular follow-ups at specified intervals. Thus, Brock's operation is an excellent preliminary palliative procedure for one-stage total correction of TOF. There is a need to revive 'Brock's procedure' for patients with TOF and poor pulmonary artery anatomy as the procedure of choice. The first direct intra-cardiac operation aimed at directly addressing the pathological anatomy on its Diamond Jubilee Year., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Bhende et al.)

At the time of writing, two patients who underwent modified Senning's operation (MSO) for the treatment of transposition of great arteries (TGAs) were followed up. At the time of surgery, the patients were three months and 15 years old, respectively. The duration of the follow-up was three years, during which there was a good prognosis, and hence no further invasive treatments were required. There was normal functioning of the right ventricle (RV) in both patients, with the exception of a minor baffle leak in the three-month-old patient. At the annual three-year follow-up, the tricuspid regurgitation (systemic atrioventricular valve) status was moderate in the three-year-old child and mild in the 18-year-old girl. Both patients maintained sinus rhythm and are assigned classification as New York Heart Association (NYHA) Classes I and II. This study aims to assess the midterm outlook after MSO in order to identify and manage future long-term complications. Our report shows a positive outcome in terms of survival and functional activities among children with d-TGA; however, there is a strong need for future research to evaluate the prognosis in the long term (LT) and to assess the functioning of RV., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Bhende et al.)

Newborns with untreated single ventricles develop pulmonary vascular diseases early in their lives. At that age, during the first eight weeks after birth, clinicians perform pulmonary artery (PA) banding to reduce the blood flow to the lung, decreasing the likelihood of future high vascular resistance or pressure. PA banding is also considered an initial stage in the process of single ventricle palliation procedures. We report a case of a 16-month-old toddler (7 kg) with room air saturation of 82%, diagnosed with tricuspid valve atresia, large atrial and ventricular septal defect, and hypoplastic right ventricle with severe pulmonary arterial hypertension. The baby underwent a successful surgical procedure of PA banding and was discharged after 13 days of hospital stay with a room air saturation of 89%. This case highlighted the benefit of PA banding beyond the stipulated period., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Bhende et al.)

Preoperative exchange transfusion is frequently recommended in patients with homozygous sickle cell anemia (homozygous SS) who undergo cardiopulmonary bypass to reduce the concentration of circulated sickle hemoglobin. The information regarding the ideal level of sickle hemoglobin for sickle cell disease (SCD) patients who require surgery is still divergent in the literature. We present the successfully managed cases of two children aged 11 months and three years with homozygous SS who underwent cardiopulmonary bypass for double-outlet right ventricle and cor-triatriatum sinistrum, respectively. In both cases, we performed preoperative blood and exchange transfusion, as well as strict intraoperative invasive monitoring. We also maintained normothermia, avoided hypoxia and acidosis, and offered effective pain management., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Soni et al.)

Myocardial infarction (MI) is typically followed by numerous lethal complications. One such complication is left ventricular free wall rupture (LVFWR). We present the case of a middle-aged hypertensive patient who had a history of unstable angina for seven days. He presented to the emergency room with chest pain, dyspnea, and unstable vital parameters. Clinical signs, electrocardiography, and echocardiography raised the suspicion of left ventricular free wall rupture with ST-segment elevation inferior wall and lateral wall MI. As a result, the patient received aggressive resuscitative measures. Later, he underwent surgical repair for cardiac rupture via cardiopulmonary bypass. Finally, the patient was discharged from the hospital on the 10 th postoperative day. The window period from the onset of cardiac wall rupture to patient admission to the operating room is crucial. This case report highlights that a high index of suspicion for left ventricle free wall rupture should be considered for a patient presenting with MI and cardiogenic shock. Timely diagnosis and quick surgical intervention can deter complications and save the patient., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Panesar et al.)

The bidirectional Glenn (BDG) or hemi-Fontan technique, performed under cardiopulmonary bypass (CPB) and often utilized as first-stage palliation for various cyanotic congenital heart diseases, as a part of the single-ventricle repair is associated with adverse side effects and high expenditures. Previous studies have shown that BDG is safe even without CPB, which thus necessitates further investigation. This manuscript presents the case of two patients with complex cyanotic congenital heart diseases. The first case was an 11-month-old baby who presented with fever, cough, and cold, while the second case was a two-year-old baby who was underweight due to poor feeding status. Both patients underwent a BDG and main pulmonary artery partial ligation following the requisite preoperative medical evaluations on CPB. Moreover, case 1 had atrial septectomy, while case 2 had significant aortopulmonary collateral arteries ligation. Both patients were discharged following an uneventful postoperative outcome. We found that an adequate selection of patients for the BDG procedure, with or without CPB, could lead to identical postoperative outcomes with regard to mortality, morbidity, and supply usage., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Bhende et al.)

Although a new right ventricle outflow can be introduced during pulmonary artery reconstruction, it is a suboptimal option as the valved conduits that mimic the natural right ventricular outflow do not grow, and a surgical conduit replacement cannot be averted. This study reported the implementation of hand-made polytetrafluoroethylene (PTFE) tricuspid-valved conduits to rebuild the right ventricular outflow tract in toddlers with truncus arteriosus and risk factors for earlier conduit explant. Herein, we described a case report of a 9-month-old toddler diagnosed in November 2021 with truncus arteriosus type I with ventricular septal defect (VSD) and severe pulmonary arterial hypertension, who has been successfully discharged 20-days postoperative surgical reconstruction with good bi-ventricular functions. Hand-made PTFE tricuspid-valved conduits are efficient in the reconstruction process of the right ventricular outflow tract in children with truncus arteriosus. The conduits are cheap, easily available, and lack potential sensitization., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Bhende et al.)

Cor triatriatum is a rare structural congenital cardiac anomaly in which one of the atria chambers is anatomically divided. If left untreated, cor triatriatum can eventually lead to heart failure. This case report describes our experience with two pediatric patients (a three-year-old girl and an 11-month-old male infant) who underwent surgical correction of cor triatriatum. Both patients underwent excision of the cor triatriatum membrane via cardiopulmonary bypass and had an uneventful postoperative recovery with good outcomes. Surgical repair of cor triatriatum sinister provides satisfactory short-term and long-term outcomes with a low risk of requiring additional intervention., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Bhende et al.)

Long-segment pulmonary atresia (PA), non-confluent branch pulmonary arteries, ventricular septal defect, tricuspid valve atresia (type 1A), and single ventricle physiology is a relatively rare and extremely heterogeneous form of congenital heart disease. This subset of patients having pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (MAPCAs) have to undergo multiple unifocalization staging operations before a complete repair is attempted. Most of the patients were deemed inoperable. We report a rare case of a concomitant single-stage unifocalization and cavopulmonary anastomosis (bi-directional Glenn procedure) in an adolescent cyanotic girl with tricuspid valve atresia (type 1 A), long-segment pulmonary atresia, non-confluent branch pulmonary arteries, bilateral patent ductus arteriosus, MAPCAs, and single-ventricle physiology. Reconstruction of the absent central pulmonary artery and non-confluent branch pulmonary arteries was achieved by dividing the bilateral patent ductus arteriosus feeding the bilateral pulmonary arteries., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Bhende et al.)

The low prevalence of scimitar syndrome along with its varied clinical presentation poses a diagnostic dilemma to the treating clinicians. It usually falls under a large spectrum of conditions called venolobar syndrome. Scimitar involves the partial venous drainage of the right lung to the inferior vena cava (IVC). We share our experience of two cases that were diagnosed as partial anomalous pulmonary venous connection/drainage (PAPVC/PAPVD) on echocardiography but CT scan revealed the underlying scimitar syndrome. Perioperative pulmonary arterial hypertension, intraoperative ventilation strategies for managing associated lung hypoplasia, and postoperative right ventricular dysfunction are a few challenges faced in the perioperative period., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Panesar et al.)

This is a case report of a child who had acyanotic congenital heart disease - ventricular septal defect (VSD) and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension. The child underwent open-heart surgery - VSD closure with PDA ligation - and six months later was re-admitted for congenital lobar emphysema of the right middle lobe. He underwent successful right middle lobectomy of the lung six months after cardiac surgery under a one-lung ventilation technique in which application of fiber-optic bronchoscope made the surgery safer and more suitable., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Bhende et al.)

This case report depicts how a case of the subretinal neovascular membrane was managed with intravitreal ranibizumab injections. A 59-year-old female patient presented with complaints of diminution of vision in her right eye for one month. Various necessary examinations were carried out and the patient was diagnosed with both forms of age-related macular degeneration (ARMD) disorder - wet ARMD in the right eye and dry ARMD in the left eye. Pseudophakia was also seen in both eyes. Drusen deposits, characteristic of the disorder, were seen in the macular area of the oculus sinister (OS). The patient was treated for the wet ARMD with intravitreal injections of 0.5 mg ranibizumab administered one month apart in the right eye. The patient showed improvements in her visual acuity and a complete resolution of the subretinal fluid., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Sharma et al.)

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that may present isolated or may be associated with other cardiac malformations. Most of the patients develop symptoms during infancy but some may remain asymptomatic up to adulthood. Symptoms range from mild distress to severe irritability and feed intolerance. We report a case of a five-month-old male child who presented with congestive heart failure and was diagnosed as a case of ALCAPA with left atrial and left ventricular dilation based on two-dimensional echocardiography and computed tomography (CT) coronary angiogram. Left main coronary artery was shown to be arising from the posteroinferior wall of main pulmonary artery. Various surgical approaches have been suggested in the repair but the Takeuchi technique was preferred owing to its origin from the non-facing sinus of the pulmonary artery and co-existing dilatation of left atria and ventricle. The surgery was uneventful and there were no postoperative complications. A cardiac CT dynamic study was also done on the follow-up visit five months later and no signs of abnormality or complications were reported. Early intervention is necessary to prevent irreversible cardiac complications and early mortality., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Bhende et al.)

Alagille syndrome (ALGS) is a rare autosomal dominant genetic disorder with multisystem involvement including the liver, heart, skeleton, eyes, kidneys, and other organ systems, along with characteristic facial abnormalities. Some patients with ALGS may have isolated involvement of a particular system, such as a heart defect like the tetralogy of Fallot, an atrial septal defect (ASD), a characteristic facial appearance, or an isolated vertebral body anomaly. These individuals may or may not have liver anomalies or other features typically seen in the disorder. We report the case of a four-year-old female child with moderate ostium secundum ASD and branch pulmonary artery stenosis diagnosed since three months of age who presented with classical features of facial dysmorphism, posterior embryotoxon in the right eye, butterfly presentation of the T5 vertebra, delayed mental development, and history of recurrent infections. Bilateral branch pulmonary artery plasty with glutaraldehyde-treated pericardial patch and direct closure of the ASD leaving a patent foramen ovale was performed to correct the cardiac malformations. The surgery was uneventful without any postoperative complications. Currently, as no curative management of the disorder is available, the syndromic medical and surgical approach remains the mainstay in managing this condition., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Bhende et al.)

Pyoderma gangrenosum (PG) is a rare ulcerating inflammatory skin condition, the cause of which remains idiopathic. A 35-year-old female presented to our outpatient department (OPD) with initial findings of multiple non-healing ulcers that developed suddenly with a symmetric bilateral pattern on the dorsal aspect of hands, elbows, and inner knees. Lesions initially developed as a pustule that progressed to burst and leave behind a raw area that turned into an ulcer. Polyarthralgia preceded these symptoms. Extensive investigations were done for the varied differentials that were postulated; this led to finally declaring PG associated with seronegative arthritis as a diagnosis of exclusion. Initial treatment with antibiotics showed little results, and thus, the patient was started on a systemic corticosteroid which proved to be successful., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Sharma et al.)

Pulmonary tuberculosis (TB) is one of the top 10 leading causes of death in the world. Multi-drug resistant TB can lead to short-term and long-term sequelae causing clinical, psychosocial, and financial burden on the diseased. Pregnancy in a woman with compromised pulmonary function is a challenge for the treating obstetrician. A multidisciplinary approach involving a respiratory physician, pre-conceptional counseling, and delivery at a tertiary care center can reduce maternal morbidity and mortality. Compliance with anti-tubercular treatment with regular follow-up can minimize the long term effects of pulmonary TB. We report a case of unilateral lung collapse due to multidrug-resistant pulmonary TB in pregnancy with good maternal and fetal outcomes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Sharma et al.)