Adolescent ; Adult ; Arteriovenous Fistula ; surgery ; therapy ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; surgery ; therapy ; Heparin ; therapeutic use ; Humans ; Infant ; Male ; Middle Aged ; Pulmonary Artery ; abnormalities ; surgery ; Pulmonary Veins ; abnormalities ; surgery ; Retrospective Studies ; Telangiectasia, Hereditary Hemorrhagic ; surgery ; therapy ; Young Adult

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital heart defect. Herein, we describe three cases of ARCAPA in an 8 months old, 18 months old, and 4 year old child. Two cases were incidentally diagnosed using a computed tomographic angiograph, and the other was incidentally diagnosed using a coronary angiograph. These cases underwent a reimplantation technique on diagnosis and resulting in positive clinical outcomes during the follow-up period which was a mean of 1.5 years.
Cardiac Surgical Procedures ; Child ; Congenital Abnormalities ; Coronary Vessel Anomalies ; Coronary Vessels* ; Diagnosis ; Electrocardiography ; Follow-Up Studies ; Heart Defects, Congenital ; Humans ; Pulmonary Artery* ; Replantation

Adult ; Arteriovenous Fistula ; diagnosis ; surgery ; Brain Abscess ; diagnosis ; surgery ; Humans ; Male ; Pulmonary Artery ; abnormalities ; surgery ; Pulmonary Veins ; abnormalities ; surgery

OBJECTIVETo review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.

METHODSThere were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.

RESULTSPostoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.

CONCLUSIONSAlthough late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.

Aged ; Aorta ; Bland White Garland Syndrome ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Heart ; Heart Failure ; Hospital Mortality ; Humans ; Mitral Valve Insufficiency ; Perioperative Period ; Postoperative Care ; Prostheses and Implants ; Pulmonary Artery ; abnormalities ; Reoperation ; Treatment Outcome ; Ventricular Function, Left

Aorta ; abnormalities ; Humans ; Pulmonary Artery ; abnormalities

A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.
Adult* ; Cardiovascular Abnormalities ; Congenital Abnormalities ; Dyspnea ; Esophagus ; Humans ; Pulmonary Artery* ; Tomography, Spiral Computed ; Trachea

Pulmonary systemic arterialization to normal basal lung without sequestration is a rare congenital anomaly. In this rare abnormality, arterialization of the left lower lobe is the most common type. In general, surgical treatments have been performed. Recently, for reducing the complications and risks of surgery, embolization is mainly attempted by using coils. We report a case of 22-year-old male patient with a 10 mm anomalous arterial supply to his normal lung, which is being successfully treated by transcatheter embolization when using the Amplatzer Vascular Plug that has been adapted for the treatment of high-flows and large artery occlusions.
Arteries ; Embolization, Therapeutic ; Humans ; Lung ; Male ; Pulmonary Artery ; Respiratory System Abnormalities ; Young Adult

OBJECTIVETo discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors.

METHODSThe clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case. In addition to 4 cases the remaining 38 patients were still combined with other cardiovascular malformations. Thirty-six cases of children underwent spiral CT airway remodeling, 23 children underwent fiber- bronchoscopy. In addition to 2 cases of airway abnormalities not seen, the rest of the children were present in varying degrees in different parts of tracheal stenosis or tracheomalacia. All patients underwent surgical correction of congenital vascular ring, concomitant heart deformity correction surgery according to the situation (7 cases of atrial septal defect repair, 3 of ventricular septal defect repair, 1 of mitral valvuloplasty, 1 of bi-Glenn, 1 of coarctation of the aorta correction with ventricular septal defect repair, 1 of trilogy of Fallot correction). One case suffered resection of tracheal stenosis and Slide tracheoplasty simultaneously, another case was implanted tracheal stenting postoperatively after pulmonary artery sling correction.

RESULTSThree patients died in hospital (7.1%), the cause of death were recurrent granuloma formation postoperatively. Remaining 39 patients were successfully discharged. The median time of overall survival of children with mechanical ventilation was 14 h (22 h) (M (Q(R))), median ICU residence time was 5 d (8 d), and the median in-hospital time was 19 d (9 d). Tracheal intubation time and postoperative ICU duration time in children with pulmonary artery sling children were much more than in children with double aortic arch (23 h (123 h) vs. 9 h(9 h), 7 d (13 d) vs. 4 d (2 d)), but the difference were not statistically significant. There was significant difference in the duration of hospitalization between the patient with the pulmonary artery sling and double aortic arch (23 d (9 d) vs. 16 d(6 d)) (χ(2) = 10.157, P = 0.006).

CONCLUSIONSThe recent results of surgical treatment of congenital vascular ring is safe and effective. The extent and scope of tracheal stenosis and tracheomalacia is a critical influence prognosis.

Aorta ; abnormalities ; Aorta, Thoracic ; abnormalities ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; surgery ; Heart Septal Defects, Ventricular ; surgery ; Heart Ventricles ; abnormalities ; Humans ; Infant ; Infant, Newborn ; Male ; Prognosis ; Pulmonary Artery ; abnormalities ; Retrospective Studies ; Tomography, Spiral Computed ; Trachea ; surgery ; Tracheal Stenosis ; surgery ; Treatment Outcome

Aorta, Thoracic ; abnormalities ; Child, Preschool ; Ductus Arteriosus, Patent ; complications ; Female ; Humans ; Pulmonary Artery ; abnormalities ; Subclavian Artery ; abnormalities

OBJECTIVETo investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).

METHODData of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up.

RESULTMost patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure.

CONCLUSIONThe accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.

Bland White Garland Syndrome ; Cardiac Output, Low ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Echocardiography ; Electrocardiography ; Extracorporeal Membrane Oxygenation ; Humans ; Infant ; Mitral Valve Insufficiency ; Perioperative Care ; methods ; Pulmonary Artery ; abnormalities ; Retrospective Studies ; Ventricular Function, Left