Since the year 2000, we have used Contegra conduits for right ventricular outflow tract reconstruction in infants and newborns. Published reports of early and late results from multiple centers have included variable and inconsistent findings. Concerns about the durability of small conduits placed in younger infants have been expressed. We report an interesting experience with a 12-mm Contegra conduit that we explanted 16 years after implantation in the course of repair of truncus arteriosus (common arterial trunk) in an infant.
Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.
We present the use of a low-resistance membrane oxygenator (Quadrox D, Maquet) in series with a pulsatile right ventricular assist device (Berlin Heart EXCOR, Berlin Heart) in a patient with biventricular support who required high-frequency oscillatory ventilation (HFOV), due to refractory acute respiratory distress syndrome associated with Cytomegalovirus pneumonia. The high mean airway pressure associated with the use of HFOV resulted in a significant negative impact on left ventricular assist device (LVAD) filling that led to a combined respiratory and metabolic acidosis and the need for vasopressor support. Oxygenator placement enabled transition to conventional ventilation and the discontinuation of vasopressor support. This case demonstrates the feasibility and safety of the use of this lung support system in patients requiring ventricular assist device (VAD) support.
We report two neonates with distal ductal origin of the left pulmonary artery who also had rare vascular rings comprised of a left aortic arch and right arterial duct with a midline/rightward descending aorta. To the best of our knowledge, this association has not previously been described, although other abnormalities of the left pulmonary artery in the setting of vascular rings have been reported. We review the embryology, utility of imaging, and clinical course.
Extracorporeal membrane oxygenation (ECMO) is an accepted treatment modality for life support refractory to conventional efforts in neonates with complex congenital heart lesions. Cannulation for ECMO can be accomplished by venovenous (VV) access where patients receive primarily respiratory support or venoarterial (VA) access which provides complete cardiopulmonary support. VV ECMO delivered by a single-vessel cannulation with a dual-chamber venous cannula allows for respiratory support while the patient remains dependent upon intrinsic cardiac function to support hemodynamics. We present a case of support of a newborn with single ventricle physiology and intraparenchymal near-atresia of the pulmonary veins using VV ECMO.
Accessory mitral valve tissue (AMVT) causing left ventricular outflow tract obstruction (LVOTO) is rare. We report a case of AMVT causing severe LVOTO resulting in acutely progressive symptoms of near-collapse. Urgent surgical resection eliminated the patient’s life-threatening symptoms. AMVT should be considered among potential LVOTO diagnoses, and early surgical intervention may be required.
Transposition of the great arteries occurs rarely in patients with mirror image dextrocardia and situs inversus, while the combination with severe coarctation of the descending aorta (CoA) makes the anatomy even more unusual. Therefore, it is not surprising that a case with such unusual and complicated anatomy presents unique problems when a primary definitive correction is attempted. We report a patient with situs inversus totalis and complex congenital heart disease including transposition of the great arteries and severe CoA who underwent successful complete, single-stage, anatomic correction.
Raghib defect and double-orifice tricuspid valve are two rare congenital heart defects. We report a case of a 42-year-old man with both Raghib defect and DOTV. The patient underwent reroofing of the coronary sinus with an intra-atrial baffle and annuloplasty of the tricuspid and mitral valves via median sternotomy.
Percutaneous pulmonary valve implantation is now an acceptable treatment option. The Edwards SAPIEN valve, intended for transcatheter aortic valve implantation, has been used in patients whose landing zone diameter is >22 to 24 mm which is not suitable for Melody valve. We report a patient in whom we successfully placed the latest generation, Edwards SAPIEN 3 (ES3) in the pulmonary position. However, because of the short covered portion of this valve, there was a significant paravalvular leak through the stent struts requiring implantation of a second ES3 valve.
To assess medium-term effects of treatment for mild to moderate recoarctation in an adult population.
We identified all patients who had coarctation repair between 1965 and 1985 in our institution. They were all (n = 156) invited to a thorough outpatient examination, which was accepted by 133. Eleven (median age 36 [28-53] years) were identified with isolated mild to moderate recoarctation and received reintervention. They comprise the patient population of the current study. We performed bicycle exercise testing, transthoracic echocardiography, magnetic resonance imaging, and 24-hour blood pressure monitoring before and 14 to 29 months after reintervention.
Compared to the values before reintervention, 24-hour blood pressures were reduced, but only significantly so for nighttime values. On echocardiography, wall thickness was reduced (1.2 ± 0.3 vs 1.1 ± 0.2, P > .05), and maximum flow velocity in the distal aortic arch decreased (2.8 ± 0.5 m/s vs 2.1 ± 0.4 m/s, P < .05). The invasive coarctation gradient was significantly reduced (15 ± 7 mm Hg vs 5 ± 9 mm Hg, P < .05), and the diameter at the coarctation site was increased (11 ± 3 mm vs 15 ± 3 mm, P < .05). Maximum achieved workload increased (182 ± 51 W vs 205 ± 65 W, P < .05). Cardiac symptoms were overall reduced.
Reintervention was overall beneficial in increasing exercise capacity and reducing symptoms in the medium term, but the effects on established myocardial hypertrophy and blood pressures were less profound. Larger studies are needed in order to establish the definite role of reintervention for patients with milder recoarctation.
We report the case of a 23-year-old man who developed constrictive pericarditis within four months after pulmonary valve replacement and repair of partial anomalous pulmonary venous connection. He had previously undergone repair of tetralogy of Fallot in infancy. After an unsuccessful trial of medical management for persistent right heart failure, magnetic resonance imaging was done, which showed a thickened pericardium. He underwent a radical pericardiectomy with a good outcome. The case is presented to illustrate a less well-recognized cause of cardiac failure following congenital cardiac surgery, which may otherwise be attributed to the failure of surgery or residual complications.
We report two cases of newborns with critical pulmonary stenosis having intact ventricular septum, who underwent pulmonary valve balloon valvuloplasty followed by banding of a patent ductus arteriosus. Transcatheter pulmonary valvuloplasty was performed one week after delivery. Following the procedure, both developed "circular shunting" as a consequence of left-to-right ductal flow and pulmonary regurgitation. This in turn caused increased blood flow into a dysfunctional right ventricle and low systemic cardiac output syndrome. The PDA banding was performed urgently as a rescue measure in order to restore systemic flow while still maintaining some duct-dependent pulmonary blood flow. This approach resolved the circular shunting. Outcome was favorable in both the patients.
A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predisposing factors. Computed tomography pulmonary angiography supported by three-dimensional reconstruction was diagnostic. The embolus qualified as massive by conventional anatomical guidelines, but as low risk by more recent functional criteria. Functional assessment has complemented morphologic assessment for risk stratification in adult patients. Such evidence is scarce in pediatrics. The patient underwent surgical embolectomy, followed by prophylactic anticoagulation, without further events. Diagnostic and management challenges are discussed.
A 2-month-old male with dysmorphic facies, neonatal thrombocytopenia, left congenital cataract, and long bone abnormalities became hypotensive with ST depression on induction of anesthesia for congenital cataract repair. Echocardiogram demonstrated decreased left ventricular function (ejection fraction 46%), mild mitral valve regurgitation (MR), and regional wall motion abnormalities. The left coronary artery could not be visualized. Subsequent cardiac catheterization confirmed atresia of the left main coronary artery. The patient underwent cardiac surgery with coronary artery bypass grafting of the left internal mammary artery to the left anterior descending coronary artery. His postoperative course was uncomplicated, his ventricular function normalized, and he was discharged home. Over the next few months, he developed progressive, severe MR refractory to medical management. Repeat cardiac catheterization revealed stenosis of the right proximal coronary artery, raising concern for progressive coronary involvement. In addition to dysmorphic features and failure to thrive, there were profound developmental delays and a finding of vacuolated lymphocytes on blood smear, which led to a diagnosis of mucolipidosis II (Inclusion [I]-cell disease). The patient was referred for mitral valve replacement, which was successful; however, ongoing respiratory issues attributed to the progression of I-cell disease led to a prolonged hospitalization with placement of a tracheostomy. Reports of coronary anomalies in patients with I-cell disease are rare. An association between mucopolysaccharidosis syndromes and coronary artery abnormalities has been established and is supported by this case report, highlighting the importance of considering the potential for coronary artery involvement with I-cell disease and other similar storage diseases.
An intramural coronary artery in the setting of truncus arteriosus (common arterial trunk) is an uncommon association. Following an uneventful surgical repair, a neonate developed a low cardiac output state deteriorating into cardiac arrest shortly after arrival into the intensive care unit, requiring extracorporeal membrane oxygenation support. Echocardiography and angiography showed occlusion of the left coronary artery, prompting emergency surgical reexploration. A "slit-like" orifice with an intramural left coronary artery was successfully unroofed, allowing full recovery. Full definition of the proximal coronary anatomy beyond the orifices should be investigated preoperatively in truncus arteriosus, as a missed intramural segment could lead to significant morbidity or mortality.
Heart failure is a common problem in the ever growing population of patients with palliated congenital heart disease. It is frequently complicated by hyponatremia that has been associated with increased morbidity and mortality. Tolvaptan is a vasopressin receptor antagonist that has been effective in improving hyponatremia and congestive symptoms in adults with chronic heart failure. We describe the short-term use of tolvaptan to treat hyponatremic hypervolemia in an adolescent patient with chronic heart failure in the setting of palliated congenital heart disease prior to definitive surgical intervention. In this case, the patient had improvement in hyponatremia and a decrease in body weight, without any adverse effects.
Aortic arch atresia has not been associated with coronary anomalies. We present a unique case of a patient with Turner syndrome and aortic arch atresia with the incidental finding of a right coronary artery to pulmonary artery fistula at the time of surgical repair.
Protein losing enteropathy (PLE) is a severe consequence of Fontan physiology that sometimes requires heart transplantation for definitive treatment. We describe transplant refractory PLE in a patient with heterotaxia and complex systemic and hepatic venous connections, successfully treated by transcatheter relief of multiple venous obstructions.
We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.
Of the associated cardiac defects with absent pulmonary valve, the combination of tricuspid atresia, ventricular septal defect, and aneurysmal dilatation of the pulmonary arteries is very rare. We report the case of a low-birth-weight girl (2,282 g) with this anomaly, which was prenatally diagnosed. Fontan completion was successfully achieved at 16 months of age, following staged palliative procedures including banding of the main pulmonary trunk and plication of the aneurysmal dilated central pulmonary artery.
We describe a case of sudden-onset left-sided hemiparesis and dysarthria in a five-year-old boy. Acute vascular malformation bleeding or ischemic stroke was suspected. Neurological examination three weeks after the initial event revealed mild residual facial paresis. Brain angiography ruled out a vascular malformation. A work-up echocardiogram revealed a 4-cm left atrial mass compatible with cardiac myxoma. Urgent surgical resection of the mass under cardiopulmonary bypass confirmed the diagnosis. Uneventful recovery followed surgical resection. In this report, we present a partially embolized left atrial myxoma that caused an acute ischemic stroke, which is rarely considered and encountered in the pediatric population.
Anomalous origin of the right coronary artery from the main pulmonary artery (anomalous right coronary artery from pulmonary artery; ARCAPA) is a rare congenital anomaly. Here, we present an unusual case of anomalous right coronary artery from the main pulmonary artery with proximal intramural course.
Loeys-Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder associated with aortic aneurysmal disease. Kommerell diverticulum (KD) is a rare aortic diverticulum, for which the indication for surgery and the surgical techniques remain subjects of debate. We describe our experience with a successful total aortic arch replacement including KD resection through a median sternotomy for a pediatric patient with LDS.
Based on described benefits of fast-tracking and early extubation in children undergoing congenital heart surgery, we applied this concept to selected children following uncomplicated orthotopic heart transplantation (OHT). In this case series, we report four patients who were extubated immediately after surgery in the operating room. A mild respiratory acidosis and hypercapnia were noted on the initial arterial blood gases, were well tolerated, and were normalized within 6 to 12 hours. There was no mortality among patients who were extubated in the operating room, and no patients required reintubation. We conclude that operating room extubation is feasible in selected patients undergoing OHT.
A 24-year-old male with past medical history of hypoplastic left heart syndrome and staged reconstructive surgery in infancy culminating in the Fontan circulation presented to the hospital with a chief complaint of chest pain described as an "elephant sitting" on his chest. Initial 12-lead electrocardiogram revealed 2-mm ST segment elevation in inferior leads, 3-mm ST-segment elevation in anterolateral precordial leads V3 and V4, and 2-mm ST-segment elevation in V5 and V6, with right axis deviation. He was transported emergently to the cardiac catheterization laboratory where coronary angiography revealed complete occlusion of multiple anomalous branches of the right coronary system with hazy appearance suggesting the presence of thrombotic material. An aspiration catheter was used successfully to reestablish TIMI grade III flow. The patient was treated with aspirin, brilinta (ticagrelor), and anticoagulation with vitamin K antagonism to prevent recurrent thromboembolic complications.
Ventricular septal defect (VSD) with valvar pulmonary stenosis (PS) is a combination of cardiac defects for which treatment by means of percutaneous catheter-directed intervention is sometimes considered. Septal occluder device embolization is a rare but potentially dreadful complication. Adequate precautions are of great importance, as operator-related and anatomical factors can contribute to the risk of device embolization. In this report, we present a case of a five-year-old patient with a perimembranous VSD and valvar PS with infundibular muscle hypertrophy. The PS was treated with balloon pulmonary valvotomy, and the VSD was closed with a catheter-directed duct occluder device. Soon after deployment, the device embolized to the aortic arch, possibly as a result of the single disc device being "milked" out of VSD by dynamic contractions of hypertrophied muscle in the right ventricular outflow tract. The embolized device was successfully retrieved and removed using cardiopulmonary bypass and a period of circulatory arrest.
Anomalous origin of the left main coronary artery from the noncoronary sinus (LCANS) is a rare variant of anomalous aortic origin of a coronary artery that is not characterized by an interarterial course. Despite the absence of an interarterial course, there are rare reports of LCANS presenting with sudden death, life-threatening arrhythmia, exercise-induced myocardial ischemia, premature ventricular contractions, and syncope. We report a patient with LCANS presenting with acute myocardial infarction who recovered completely with emergent surgical unroofing of the coronary artery.
Infective endocarditis (IE) is an uncommon condition among patients with congenital heart disease, however it can be life threatening. The usual management includes replacement of the affected valve, especially in patients with aortic valve compromise, and is even more common in previously repaired valves. In this case report, we describe the successful reconstruction of an aortic root destroyed by IE, in a patient with history of ballooning of a congenital aortic stenosis.
Survival is significantly compromised in infants born with congenital diaphragmatic hernia and major cardiac anomalies. Mortality is highest when congenital diaphragmatic hernia occurs in association with d-transposition of the great arteries. We present three infants with congenital diaphragmatic hernia associated with d-transposition of the great arteries from a single institution. All three infants survived to discharge after surgical repair/palliation of both the diaphragmatic hernia and heart defect and are doing well at last follow-up. The clinical course and management of these three patients are described.
We report the use of video-assisted cardioscopy (VAC) to assess the complete resection of a giant sessile rhabdomyoma of the left ventricle (LV) in a case of suspected neonatal tuberous sclerosis. A fetal echocardiogram performed at 20 weeks of gestation identified the mass at the apex of the LV and attached to the interventricular septum (IVS). Further echocardiography during the pregnancy demonstrated moderate growth of the LV mass. This was occupying more than two-thirds of the LV cavity after the delivery, and there were concerns of LV outflow tract obstruction. The 4-kg newborn underwent LV tumor excision via a left atrial approach at the age of 12 days. The mitral valve (MV) apparatus and the IVS had strong attachments to the mass. The tumor was successfully resected. In view of the invasiveness of the tumor, a decision was taken to perform an intracardiac assessment using a VAC to detect any iatrogenic complication. A Karl Storz Neuro Endoscope probe 4 mm in diameter and 14 mm in length with an angulation of 0° was passed into the left ventricular cavity through the MV with the aim to detect residual tumor and to identify any iatrogenic complications due to the extensive resection. Video-assisted cardioscopy allows visualization of inaccessible intraventricular structures avoiding ventriculotomy, which can cause ventricular dysfunction, arrhythmias, and aneurysm formation. The VAC was shown to be very useful in this situation, and the process can easily be repeated as required.
Giant congenital right atrial aneurysms are rare, with their natural history complicated by death, heart failure, arrhythmias, and thrombosis. Prenatal diagnosis of the defect is feasible and allows an early preparation of a plan for management. We present details of a patient diagnosed prenatally with a giant right atrial aneurysm, which was successfully treated surgically as soon as the patient became symptomatic.
When the interventricular septum is intact, a well-formed nonsystemic ventricle with no outlet is prone to hypertension and hypertrophy after Fontan operation. Herein, we report a patient who had a complex d-transposition of the great arteries palliated over 14 years ago with a Fontan operation and presented with a hypertensive left ventricle (LV) contributing to heart failure. He underwent surgical creation of a ventricular septal defect to decompress the LV and was able to resume his desired lifestyle.
We describe a neonate with severe left ventricular (LV) dysfunction manifesting soon after birth, who required extracorporeal life support (ECLS). Multiple attempts at separation from ECLS resulted in high left atrial pressures and hemorrhagic pulmonary edema. The inability to separate from ECLS after ten days prompted an unconventional treatment strategy. We performed a modified Norwood operation for LV rehabilitation on day 10 of ECLS. This was followed by successful separation from ECLS. Left ventricular function recovered, and the child underwent conversion to a biventricular circulation on 128 days post-Norwood.
Endocarditis is a consideration in the differential diagnosis when masses are seen on echocardiography in a patient with congenital heart disease. We present a case of insidious development of endocarditis caused by Streptobacillus moniliformis in a seven-month-old baby after a rat bite, when the baby was three months of age.
Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state. Right heart dysfunction in infants can result in severe conjugated bilirubin, likely as a consequence of venous congestion and can be used as an indirect marker of right heart dynamics
Cardiac fibroma is a rare disease, and the management of asymptomatic patients is controversial. We report a case of successful surgical excision of a large cardiac fibroma in an asymptomatic child. Surgery should be considered for such a patient, as sudden cardiac death may occur in the absence of premonitory symptoms.
A term neonate was cannulated for venoarterial extracorporeal life support (ECLS) via the right neck for non-postoperative junctional ectopic tachycardia. Initial echocardiogram demonstrated an echogenic strand in the transverse arch. Computed tomography angiogram confirmed arterial dissection of the right common carotid artery that extended into the proximal transverse arch. Dissection flap was repaired at the time of ECLS decannulation without cardiopulmonary bypass. Follow-up computed tomography angiogram revealed a segment of narrowing of approximately 50% of the right common carotid artery without false lumen or aneurysm.
We describe a case of fatal acute appendicitis in a neonate associated with congenital cardiac disease requiring staged surgery. Neonatal appendicitis is extremely rare and usually associated with prematurity and congenital abdominal conditions. This report serves to highlight the risk of this disease due to vascular insufficiency and ischemia in neonates with congenital cardiac disease and highlight the importance of considering this diagnosis in such a neonate with unexplained sepsis even in the absence of clear abdominal signs.
Tracheal obstruction secondary to vascular and soft tissue compression, after Nikaidoh procedure, can effectively be managed with aortopexy from a suprasternal incision.
Neonatal arterial switch operation for simple dextro-transposition of the great arteries (d-TGA) has almost eliminated the occurrence of pulmonary vascular obstructive disease compared to patients who underwent Mustard or Senning procedure at an older age. We report a case of a neonate with d-TGA and intact ventricular septum who underwent arterial switch operation and yet developed severe pulmonary vascular obstructive disease within two months.
Intramyocardial hematoma is a rare condition and is an incomplete form of myocardial rupture, which may occur after myocardial infarction, cardiac surgery, trauma, percutaneous coronary intervention, or spontaneously. We describe a case of a 16-year-old girl with intramyocardial hematoma mimicking an intracavitary thrombus following repair of Ebstein anomaly. The intramyocardial hematoma was incorrectly diagnosed on echocardiography as a right ventricular thrombus, and the true nature of the lesion was only realized during repeat surgical intervention for severe tricuspid regurgitation. The hematoma was managed conservatively and spontaneously resolved.
We present a six-year follow-up of an infant who underwent a left subclavian artery to left anterior descending artery anastomosis for anomalous origin of the left coronary artery from the pulmonary artery, without the use of cardiopulmonary bypass. The clinical status, echocardiography, angiography, and exercise tolerance with treadmill test were found to be normal. This technique could be a viable alternative to the more established coronary translocation, with lesser morbidity, shorter hospital stay, and lower economic burden, especially in the developing world.
Congenital left ventricular diverticulum is an extremely rare heart defect. Here, we report a case of successful endoventriculoplasty using a Dacron patch in an infant who was followed up for one year.
Arterial tortuosity syndrome is a rare autosomal recessive connective tissue disease characterized by elongation, tortuosity, and aneurysmal formation of the large and middle-sized arteries sometimes associated with stenosis of the pulmonary arteries and/or aorta. We present three cases of severe arterial tortuosity with different manifestations. In two cases, the aortic arch was involved. Angiography showed a very tortuous aortic arch, with many loops and twists and a normal descending aorta. One required operation. The third case presented multiple severe stenoses of both pulmonary arteries with many tortuous segments. Surgical repair consisted of pulmonary artery augmentation utilizing a bovine pericardial patch from hilum to hilum.
Over the last two decades, numerous conduit options and implantation techniques have been described for right ventricular outflow tract (RVOT) reconstruction in the management of tetralogy of Fallot (TOF) with hypoplastic pulmonary annulus. The limited availability of homografts and the cost factor have led us to explore the use of decellularized xenografts as an alternative. Here we present a nine-year follow-up of an adult patient with TOF with hypoplastic pulmonary annulus, who underwent reconstruction of the RVOT by the double-barrel technique, using a decellularized porcine pulmonary artery xenograft valved conduit.
Liver stiffness (LS) is associated with the presence of fibrosis; however, hepatic congestion due to elevated central venous pressure has also been shown to correlate with LS values. We report here the case of a 35-year-old woman with operated pulmonary stenosis and chronic pericarditis in whom longitudinal follow-up of LS was correlated with the changes in her hemodynamic conditions before and after surgical or percutaneous treatment of residual lesions. This case highlights the potential interest of LS as a reliable marker of hepatic congestion and elevated right ventricle (RV) filling pressures.
A neonate was diagnosed with a mediastinal mass after presenting with bradycardia. At surgery, she was found to have a 4-cm mass replacing most of the right atrial free wall. After tumor resection, the right atrium was reconstructed with an extracellular matrix biomaterial that supports native tissue regeneration. Her pathology revealed rhabdomyoma, which is rare in patients without tuberous sclerosis. The procedure was well tolerated but was complicated by narrowing of the superior vena cava that required dilation postoperatively.
Atresia of the left coronary artery ostium is extremely rare. We report the case of a 13-year-old boy who played volleyball in school and collapsed with severe chest pain during practice. He was referred to our hospital, and imaging modalities showed atresia of the left main coronary artery ostium. Urgent coronary ostioplasty was performed using a patch of 0.6% glutaraldehyde-treated autologous pericardium. His postoperative course was uneventful, and he has had a normal everyday life without chest pain 8 months postoperatively. Physicians should be aware of the patient’s history, as in this case, because prompt imaging diagnosis is essential when there is a high likelihood that the event is related to myocardial ischemia. Since long-term outcome is uncertain even after successful surgical revascularization, close follow-up is required.
We describe the diagnosis and surgical repair of a five-month-old infant with a congenital discontinuous right pulmonary artery. Initial echocardiogram failed to show the right pulmonary artery and revealed systemic left pulmonary artery pressure based on the tricuspid regurgitation jet. Computed tomographic angiography confirmed the diagnosis of discontinuous right pulmonary artery. The right pulmonary artery appeared essentially normal in size, and there were no significant aortopulmonary collateral arteries. Using cardiopulmonary bypass and aortic transection, we created an anastomosis between the right and the main pulmonary arteries augmented anteriorly by a pericardial patch. Postoperative lung perfusion scan demonstrated balanced pulmonary blood flow to the lungs. Pulmonary hypertension resolved over three weeks in the postoperative period, an expected outcome in this age-group.
Giant right atrial aneurysm is a rare entity in infants and children. It needs to be distinguished from an atrial diverticulum, which can have similar presentation. Generally, an incidental finding in children, it can present with varied symptoms. We report a case of a giant right atrial aneurysm in an asymptomatic child with a large clot in the dilated right atrium, who underwent successful resection of the atrial aneurysm.
The surgical closure of secundum atrial septal defect (ASD) is frequently performed. The minimally invasive approach, currently adopted by many centers as a way to preserve aesthetic appearance, particularly in female patients, seems to be safe. We describe the case of an adolescent who developed a desmoid tumor soon after a minimally invasive operation for surgical closure of a secundum ASD.
Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.
Cardiac foreign bodies are rare in children and the nature or timing of the injury may be hard to ascertain. We report a case of a 10-year-old boy who presented with a 28-mm solid core needle in the left chest wall that advanced into his right ventricle, possibly from soft tissue manipulation. Computed tomography, intraoperative fluoroscopy, and echocardiography were necessary to locate the needle and successfully remove it surgically.
Isolated subclavian artery arising from the pulmonary artery is exceedingly rare. Most cases are associated with other forms of congenital heart disease and typically present with subclavian or pulmonary steal syndromes. We report the case of a patient with pulmonary hypertension which masked the isolated subclavian artery, allowing it to masquerade as another common congenital heart lesion.
We present a case with left isomerism, interruption of the inferior caval vein, anomalous systemic venous drainage with partially anomalous pulmonary venous drainage, and a sinus venosus type of atrial septal defect in the absence of a right superior caval vein. This report is of interest because of a rare combination of these anomalies, which was accurately diagnosed and successfully repaired.
Neonatal critical aortic stenosis is life threatening and requires intervention shortly after birth. Early percutaneous aortic valvuloplasty is an effective way to obtain reliable prograde flow. We describe the integrated use of hybrid, open surgical, and single-ventricle rehabilitation techniques to successfully treat a neonate with borderline left-sided cardiac structures, who sustained a complication from balloon aortic valvuloplasty.
We report an uncommon case of large ostium secundum atrial septal defect (ASD) with severe pulmonary arterial hypertension causing aneurysmal dilatation of the pulmonary arteries (PAs) leading to compression of the left main bronchus and collapse of the entire left lung in a 15-month-old female child. The patient was managed by surgical closure of the ASD, translocation of the right PA anterior to the aorta with PA aneurysmorrhaphy. Left bronchial compression was relieved with complete lung expansion on the third postoperative day.
A rare variant of totally anomalous pulmonary venous connection with obliterated infradiaphragmatic vertical vein is described. A 20-day-old male child weighing 2.2 kg was admitted with cyanosis and tachypnea. Pulmonary venous return was found to be entirely through a tortuous pulmonary-to-systemic venous collateral channel, as the descending vertical vein ended blindly.
We present the case of a two-month-old male with congenital Gerbode defect, hypoplastic aortic arch, and left-sided partially anomalous pulmonary venous return. The patient underwent single-stage surgical repair, which consisted of aortic arch advancement with resection of the coarctation segment, pulmonary vein repair, and primary closure of the Gerbode defect. The anomalous pulmonary vein posed a particular challenge due to its size and distance from the left atrium, which we approached with a posterior atrial wall trapdoor baffle technique, without mobilizing the affected vein. Postoperatively and at one year follow-up, there was no evidence of residual lesions and there was unobstructed flow pattern across the aortic arch and the affected pulmonary vein.
One of the most rare and dangerous complications of a modified Blalock-Taussig shunt involves the formation of a pseudoaneurysm. A pseudoaneurysm may rupture or may result in other adverse events including compression of mediastinal structures or collapse of the underlying lung parenchyma. Shunt-related pseudoaneurysm may be associated with shunt occlusion, bacteremia, or pulmonary hemorrhage. We describe two cases complicated by large pseudoaneurysm formation, following systemic-to-pulmonary artery shunt operations. The patients in question had recurrent pulmonary hemorrhage. Both underwent successful placement of covered coronary artery stents.
We describe a 13-month-old patient with tetralogy of Fallot, left pulmonary artery discontinuity, and supracardiac totally anomalous pulmonary venous drainage to the azygos vein. The diagnosis and the management of this rare association are briefly discussed.
A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.