ABSTRACT
Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.
Subject(s)
Predictive Value of Tests , Scimitar Syndrome , Tertiary Care Centers , Humans , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/surgery , Scimitar Syndrome/physiopathology , Scimitar Syndrome/mortality , Scimitar Syndrome/therapy , Female , Male , Colombia , Child, Preschool , Child , Infant , Adolescent , Treatment Outcome , Infant, Newborn , Longitudinal Studies , Time Factors , Retrospective Studies , Pulmonary Circulation , Cardiac Surgical ProceduresABSTRACT
Scimitar syndrome is a congenital malformation that is usually associated with hypoplasia of the right lung with an abnormal blood supply by direct branches of the aorta. These branches normally require embolization.
Subject(s)
Embolization, Therapeutic , Esophageal Perforation , Scimitar Syndrome , Embolization, Therapeutic/adverse effects , Humans , Lung , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/therapyABSTRACT
Cardiac resynchronization therapy with His-bundle pacing is evolving rapidly as a viable cardiac device strategy for the treatment of severe chronic heart failure. The success of this technique in patients with congenital heart disease is facilitated by advanced integrated imaging modalities. We report a case of cardiac resynchronization therapy with His-bundle pacing with defibrillator for the management of a patient with heart failure with severely reduced ejection fraction, left bundle branch block, and congenital heart disease characterized by Scimitar syndrome with cardiac dextroposition. We highlight the contribution of integrated imaging modalities to guide accurate lead positioning.
Subject(s)
Bundle-Branch Block/therapy , Cardiac Resynchronization Therapy/methods , Heart Failure/therapy , Scimitar Syndrome/therapy , Aged , Bundle of His , Bundle-Branch Block/diagnostic imaging , Computed Tomography Angiography , Electrocardiography , Female , Heart Failure/diagnostic imaging , Humans , Scimitar Syndrome/diagnostic imagingABSTRACT
An open sternal wound is a dreaded complication after open heart surgery for neonatal congenital cardiac anomalies. Vascularised muscle flap reconstruction of sternal wound defects, to prevent life-threatening mediastinal infections, is the standard of care in adults and children. However, there is paucity of published literature regarding the safety of this technique in neonates. We describe a successful operative technique for complex reconstruction of an open heart sternal defect on a neonatal male patient. On 6 months postoperative follow-up, we identified an issue with sternal instability. Patient underwent a subsequent operation for reinforcement of the sternal wound repair with Vicryl mesh. The authors report safety of using three separate vascularised muscle flaps in a single neonatal operation. Long-term follow-up of the sternal wound reconstruction is warranted to determine need for secondary procedures.
Subject(s)
Cardiac Surgical Procedures/adverse effects , Plastic Surgery Procedures/methods , Scimitar Syndrome/therapy , Sternotomy/adverse effects , Surgical Wound/therapy , Cardiac Surgical Procedures/methods , Extracorporeal Membrane Oxygenation , Humans , Infant, Newborn , Male , Negative-Pressure Wound Therapy , Pectoralis Muscles/transplantation , Plastic Surgery Procedures/adverse effects , Reoperation/methods , Sternum/surgery , Surgical Flaps/adverse effects , Surgical Flaps/transplantation , Surgical Wound/etiology , Surgical Wound Infection/etiology , Surgical Wound Infection/prevention & controlABSTRACT
No disponible
Subject(s)
Humans , Male , Infant, Newborn , Infant , Palliative Care/methods , Scimitar Syndrome/therapy , Scimitar Syndrome/diagnosisABSTRACT
Heart failure is the main cause of death in patients with pulmonary arterial hypertension and congenital heart disease. We used an original approach in a 15-year-old girl with rapidly progressive right heart failure secondary to severe pulmonary arterial hypertension and partial anomalous pulmonary venous return. After surgical congenital heart defect repair on cardiopulmonary bypass, she was weaned off bypass using a central Novalung for 11 days, then started on triple specific pulmonary vasodilator therapy.
Subject(s)
Lung Transplantation , Preoperative Care/methods , Pulmonary Arterial Hypertension/therapy , Scimitar Syndrome/therapy , Adolescent , Female , Humans , Pulmonary Arterial Hypertension/complications , Scimitar Syndrome/complicationsSubject(s)
Palliative Care/methods , Scimitar Syndrome/therapy , Humans , Infant , Infant, Newborn , Male , Scimitar Syndrome/diagnosisABSTRACT
Objective: To characterize the clinical features and outcomes of scimitar syndrome (SS) to aid the understanding of this syndrome. Methods: This retrospective study included 6 children who were diagnosed with SS at the pediatric cardiovascular center of Beijing Anzhen Hospital from January 2012 to September 2018. SS was diagnosed by echocardiography and confirmed by cardiac computed tomography angiography(CTA) or surgery. All data were collected to analyze the clinical and imaging characteristics and prognosis. Results: Among the 6 SS children (aged 2 months to 15 years; 5 males) weighed 5.6-17.1 kg. Three cases were infant type, the clinical manifestations were recurrent respiratory tract infection with growth retardation, including 2 cases with severe pulmonary hypertension, while 3 cases with adult type, were asymptomatic. Cardiac CTA imaging showed that the right single or all pulmonary veins descended through the diaphragm and converged into the inferior vena cava. One case was isolated infracardiac partial anomalous pulmonary venous connection (PAPVC) without other malformations. The remaining 5 cases complicated with atrial septal defect, different vascular and trachea malformations as well as spinal malformations. Vascular malformations included pulmonary veins stenosis, abnormal origin of pulmonary artery branches, collateral branches of systemic artery supplying local lung tissue, and persistent left superior vena cava. The treatment varied according to the specific location of anomalous pulmonary venous connection, the degree of pulmonary hypertension and the severity of clinical symptoms. Four cases underwent one-stage radical surgery, one case accepted intervention to occlude the collateral artery which was supplying the right lower lung and received stage â ¡ radical surgery half a year later, and the remaining one case died from pulmonary hypertension crisis preoperation. Conclusions: Isolated SS can easily miss diagnosis due to mild clinical symptoms. Patients with complicated malformations can benefit from combination therapy. SS associated with severe pulmonary hypertension can lead to early death. Therefore, early diagnosis and appropriate treatment can improve the prognosis of patients.
Subject(s)
Abnormalities, Multiple/diagnosis , Scimitar Syndrome/diagnosis , Adolescent , Child , Child, Preschool , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Heart Septal Defects, Atrial/complications , Humans , Infant , Male , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Respiratory Tract Infections/complications , Retrospective Studies , Scimitar Syndrome/etiology , Scimitar Syndrome/mortality , Scimitar Syndrome/therapySubject(s)
Embolization, Therapeutic , Heart Failure/etiology , Scimitar Syndrome/therapy , Veins/abnormalities , Adult , Computed Tomography Angiography , Female , Heart Failure/diagnostic imaging , Heart Failure/physiopathology , Humans , Magnetic Resonance Angiography , Phlebography/methods , Recovery of Function , Scimitar Syndrome/complications , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/physiopathology , Treatment Outcome , Veins/diagnostic imaging , Veins/physiopathologyABSTRACT
BACKGROUND: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. METHODS: Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively. The study protocol IRB-AAAO1805 was approved. RESULTS: Forty-seven patients were identified, including 20 infants with significant associated congenital heart defects (42.6%, including 7 with single ventricle physiology), and 10 infants (21.3%) and 16 noninfants (34.0%) with isolated scimitar syndrome. Median follow-up was 3.55 years. Noninfants exhibited lower incidences of right pulmonary artery hypoplasia (p < 0.001), aortopulmonary collaterals (p = 0.004), and scimitar vein obstruction at the caval confluence (p = 0.032). Eighteen patients (38.3%) underwent surgical repair for scimitar syndrome. Overall mortality after baffle repair or scimitar vein reimplantation was 37.5% (3 of 8) for infants and 0% (0 of 6) for noninfants (p = 0.209). Overall mortality for medically managed infants was 46.7% (7 of 15) compared with 0% (0 of 8) for noninfants (p = 0.052). Multivariable analyses identified infantile onset as an independent risk factor for stenosis or obstruction after repair (hazard ratio 9.34, p = 0.048), and single ventricle physiology as an independent risk factor for mortality among unrepaired patients (hazard ratio 29.8, p = 0.004). CONCLUSIONS: The severity of scimitar syndrome depends on presenting age and associated congenital heart disease. Nonsurgical and surgical outcomes are suboptimal for infantile disease, which is a risk factor for stenosis after repair. Single ventricle physiology is associated with poor prognosis.
Subject(s)
Risk Assessment/methods , Scimitar Syndrome/diagnosis , Adolescent , Adult , Age Distribution , Child , Child, Preschool , Echocardiography , Female , Follow-Up Studies , Humans , Incidence , Infant , Infant, Newborn , Male , Middle Aged , New York/epidemiology , Retrospective Studies , Risk Factors , Scimitar Syndrome/epidemiology , Scimitar Syndrome/therapy , Survival Rate/trends , Time Factors , Young AdultABSTRACT
Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC), in which all or part of the right lung is drained by right pulmonary veins that anomalously connect to the inferior vena cava (IVC). The affected lung and its associated airways are often hypoplastic. In addition, aortopulmonary collateral vessels may be involved on the affected side, causing sequestration of that side; such involvement is commonly associated with cardiac defects. We report a case of infantile scimitar syndrome that involved a typical association with the right lung, but with extremely unusual associations with congenital hydrocephalus and heart blockage. The presentation of this case and the role of different diagnostic approaches and management are discussed.
Subject(s)
Hydrocephalus/diagnosis , Scimitar Syndrome/diagnosis , Cardiac Catheterization , Humans , Infant, Newborn , Male , Scimitar Syndrome/therapyABSTRACT
Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all (total form) or some (partial form) pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium. The authors present one case of total anomalous pulmonary venous connection and two cases of partial anomalous pulmonary venous connection, one of supracardiac drainage into the brachiocephalic vein, and the other of infracardiac anomalous venous drainage (scimitar syndrome). Through the presentation of these cases, this article aims to review the main pulmonary venous developmental defects, highlighting the role of imaging techniques in the assessment of these anomalies.
Subject(s)
Pulmonary Veins/abnormalities , Scimitar Syndrome/diagnostic imaging , Adolescent , Child , Female , Heart Atria , Humans , Incidental Findings , Infant , Magnetic Resonance Imaging , Male , Pulmonary Veins/diagnostic imaging , Scimitar Syndrome/therapy , Tomography, X-Ray ComputedABSTRACT
Scimitar syndrome is a rare congenital heart disease characterised by anomalous pulmonary venous drainage to the inferior vena cava, aortopulmonary collaterals, hypoplasia of the right lung and intracardiac defects. Surgical correction remains the gold-standard therapy. However, non-surgical intervention has been reported effective in selected cases with scimitar syndrome. We report on a one-year-old boy with scimitar syndrome who underwent stepwise transcatheter intervention as an alternative treatment. Embolisation of the aortopulmonary collaterals and occlusion of the atrial septal defect were performed using detachable coils and an Amplatzer septal occluder, respectively. The patient's postcathetherisation course was uneventful. The right cardiac chamber and pulmonary arterial pressure returned to normal during follow up.
Subject(s)
Cardiac Catheterization , Embolization, Therapeutic , Heart Septal Defects, Atrial/therapy , Pulmonary Artery/physiopathology , Pulmonary Circulation , Pulmonary Veins/physiopathology , Scimitar Syndrome/therapy , Aorta/physiopathology , Arterial Pressure , Cardiac Catheterization/instrumentation , Collateral Circulation , Computed Tomography Angiography , Heart Septal Defects, Atrial/diagnostic imaging , Heart Septal Defects, Atrial/physiopathology , Humans , Infant , Male , Phlebography/methods , Pulmonary Artery/diagnostic imaging , Pulmonary Veins/abnormalities , Pulmonary Veins/diagnostic imaging , Recovery of Function , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/physiopathology , Septal Occluder Device , Treatment OutcomeABSTRACT
Scimitar syndrome (SS) is a rare congenital malformation with an estimated incidence of approximately 2 in 100 000 births. A wide clinical spectrum is observed in children with this syndrome. The common clinical presentation in infancy is respiratory distress and tachypnoea due to associated pulmonary hypoplasia, pulmonary overcirculation and/or pulmonary hypertension. Babies with SS presenting with cardiac failure are prone to develop exaggerated pulmonary vascular disease. Hence early intervention, using either coil embolisation or surgical intervention, is indicated. We are reporting a case of a term baby boy who presented with respiratory failure during the first 24 h of life. Echocardiogram and CT angiogram revealed SS. The baby needed intubation due to respiratory failure. Aortopulmonary collaterals, identified on aortic angiogram, were successfully occluded with detachable coils.
Subject(s)
Cardiac Catheterization , Heart Failure/diagnostic imaging , Hypertension, Pulmonary/diagnosis , Pulmonary Artery/diagnostic imaging , Respiratory Distress Syndrome, Newborn/etiology , Scimitar Syndrome/diagnosis , Cardiac Catheterization/methods , Collateral Circulation , Coronary Angiography , Diuretics/administration & dosage , Echocardiography , Furosemide/administration & dosage , Heart Failure/etiology , Heart Failure/therapy , Humans , Hypertension, Pulmonary/therapy , Infant, Newborn , Male , Pulmonary Artery/abnormalities , Pulmonary Artery/physiopathology , Scimitar Syndrome/physiopathology , Scimitar Syndrome/therapy , Treatment OutcomeABSTRACT
We describe a case of a scimitar syndrome "variant" where dual drainage existed from the right upper and middle pulmonary veins to the inferior vena cava and left atrium. Device closure of the anomalous vein at the level of the connection to the IVC was successful in achieving diversion of pulmonary venous flow to the left atrium. Vigilance during work-up of anomalous pulmonary venous drainage (whether isolated or associated with other cardiac defects that may be amenable to device closure) is important to define the presence of dual connections to the left atrium, in which case a less-invasive transcatheter approach may be feasible.
Subject(s)
Cardiac Catheterization/instrumentation , Heart Atria/abnormalities , Pulmonary Veins/abnormalities , Scimitar Syndrome/therapy , Adult , Coronary Angiography , Coronary Circulation , Female , Heart Atria/physiopathology , Humans , Prosthesis Design , Prosthesis Implantation/instrumentation , Pulmonary Circulation , Pulmonary Veins/physiopathology , Scimitar Syndrome/diagnosis , Scimitar Syndrome/physiopathology , Treatment Outcome , Vena Cava, Inferior/abnormalities , Vena Cava, Inferior/physiopathologyABSTRACT
A very low birth weight neonate (1.2 kg) with total anomalous pulmonary venous connection and obstructed infracardiac pulmonary venous drainage was treated by stenting of the ductus venosus via a transjugular approach. With one reintervention to dilate an in-stent stenosis, the palliative stented situation stabilized the patient for as long as three months until definitive surgical correction at a weight of 2 kg. © 2014 Wiley Periodicals, Inc.
Subject(s)
Infant, Very Low Birth Weight , Palliative Care/methods , Pulmonary Veins/abnormalities , Scimitar Syndrome/therapy , Stents , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/surgery , Cardiac Catheterization/methods , Constriction, Pathologic/therapy , Diaphragm/abnormalities , Emergency Treatment/methods , Female , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Humans , Infant, Newborn , Risk Assessment , Scimitar Syndrome/diagnostic imaging , Treatment Outcome , UltrasonographyABSTRACT
We present the case of a 3-year-old boy with asplenia-type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left-sided scimitar vein. This pattern of pulmonary venous return has not been previously reported in a patient with asplenia. MRI x-ray fusion was used to guide transcatheter device occlusion of the scimitar vein, resulting in marked clinical improvement.
Subject(s)
Cardiac Catheterization/instrumentation , Cyanosis/etiology , Fontan Procedure/adverse effects , Heart Ventricles/surgery , Magnetic Resonance Angiography , Magnetic Resonance Imaging, Interventional , Radiography, Interventional , Scimitar Syndrome/therapy , Abnormalities, Multiple , Child, Preschool , Coronary Circulation , Cyanosis/diagnosis , Cyanosis/physiopathology , Heart Ventricles/abnormalities , Heart Ventricles/physiopathology , Hemodynamics , Heterotaxy Syndrome/complications , Humans , Male , Multimodal Imaging , Predictive Value of Tests , Pulmonary Circulation , Scimitar Syndrome/complications , Scimitar Syndrome/diagnosis , Scimitar Syndrome/physiopathology , Time Factors , Treatment OutcomeABSTRACT
The diagnosis and management of pediatric cardiac emergencies can be challenging and complicated. Early presentations are usually the result of ductal-dependent lesions and appear with cyanosis and shock. Later presentations are the result of volume overload or pump failure and present with signs of congestive heart failure. Acquired diseases also present as congestive heart failure or arrhythmias.