Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.
Adult ; Aortic Valve Stenosis/*complications/diagnosis ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Humans ; Hypoplastic Left Heart Syndrome/*complications/diagnosis ; Magnetic Resonance Imaging, Cine ; Male ; Pulmonary Valve Stenosis/*complications/diagnosis

OBJECTIVETo introduce the experience of diagnosis and surgical treatment of Williams syndrome combined with cardiovascular disease.

METHODSBetween October 1996 and June 2003, 8 patients of Williams syndrome with cardiovascular disease were admitted in Fuwai hospital. Seven patients underwent surgical correction. One didn't undergo surgical procedure. There were 6 male and 2 female ranging from 1.5 to 12.0 years old (medium age 6.4). Three had localized type supravalvular aortic stenosis and 5 diffused type supravalvular aortic stenosis. In them, 2 patients were combined with peripheral pulmonary stenosis. Single patch aortoplasty were performed in 6 cases, and inverted bifurcated patch aortoplasty in one patient.

RESULTSOne patient died and one patient suffered renal insufficiency. In the early postoperative period, the mean speed of flow was reduced to 1.7 m/s from 4.6 m/s, and the mean systolic pressure gradient was reduced from 91 mm Hg to 18 mm Hg. Six patients were followed up 16 to 91 months. There were 5 cases in NYHA function class I, and one in class II.

CONCLUSIONSatisfied result can be achieved in surgical treatment of Williams syndrome with supravalvular aortic stenosis, but it is not in combined with peripheral pulmonary stenosis.

Aortic Stenosis, Supravalvular ; complications ; congenital ; surgery ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; complications ; congenital ; surgery ; Treatment Outcome ; Williams Syndrome ; complications ; surgery

A coronary arteriovenous fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common anomaly that can affect coronary perfusion. Bilateral involvement of coronary fistula, however, constitutes an uncommon subgroup of coronary arteriovenous fistulas. We report a case which shows a rare occurrence of bilateral coronary arteriovenous fistula coexistent with atrial septal defect and pulmonic stenosis.
Arteries/abnormalities ; Arteriovenous Fistula/complications* ; Case Report ; Coronary Vessel Anomalies/complications* ; Heart Septal Defects, Atrial/complications* ; Human ; Male ; Middle Age ; Pulmonary Valve Stenosis/complications* ; Veins/abnormalities

BACKGROUND: Twelve patients with acyanotic tetralogy of Fallot (TOF), characterized by the combination of a malaligned ventricular septal defect (VSD) and infundibular pulmonic stenosis with the clinical finding of acyanosis at rest, underwent surgical correction between January 1988 and July 1997. MATERIALS AND METHODS: 9.92% of patients with the diagnosis of TOF were acyanotic TOF in the same period. Ages ranged from 12 to 42 months (mean 25.2 months). 2D-echocardiographic studies, cardiac catheterization, and angiocardiograms were performed in all patients before operation. The preoperative mean systemic arterial oxygen saturation was 93.5%. According to the 2D-echocardiographic analysis, there was Lt-to-Rt shunt through VSD in 4 patients, bidirectional shunt in 2 patients, and no shunt in 6 patients. RESULTS: The preoperative mean right ventricle to pulmonary artery (RV-PA) pressure gradients were 52.3 mmHg on 2D-echocardiogram and 48.4 mmHg on cardiac catheterization. The repair of ventricular septal defect was performed through a right atrial approach and the hypertrophic infundibular muscle bundles were resected by the transatrial and transpulmonary approach. Six patients (50%) received a transannular patch. The mean cardiopulmonary bypass time was 135.0 minutes, and the aortic crossclamp time was 87.8 minutes. Postoperative complications included bleeding necessitating reentry in one and chylothorax in one. No patient died after operation and there were no late deaths. Postoperative 2D-echocardiograms revealed tiny patch dehiscence in 5 cases and a moderate RV-PA pressure gradients (mean 15.3 mmHg). All patients were in New York Heart Association functional class 1 after operation. CONCLUSIONS: acyanotic TOF is the uncommon form of TOF, and acyanotic TOF can be repaired with a good outcome.
Cardiac Catheterization ; Cardiac Catheters ; Cardiopulmonary Bypass ; Chylothorax ; Diagnosis ; Heart ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Hemorrhage ; Humans ; Oxygen ; Postoperative Complications ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Tetralogy of Fallot*

OBJECTIVEAlthough NuMED Cheatham-Platinum (CP) stent was specifically designed to treat vascular obstructions associated with congenital heart disease (CHD), its application in pediatric patients is relatively uncommon, especially in the pulmonary artery stenosis. The aim of this study was to evaluate the immediate-, early- and intermediate-term results of CP stent implantation in the treatment of vessel stenosis associated with CHD in children and adolescents.

METHODSFrom August 2005 to May 2007, 5 consecutive patients (3 boys and 2 girls) diagnosed as vascular stenosis associated with CHD underwent CP stent implantation in our institution. One patient had native coarctation of the aorta (CoA) and four patients had pulmonary artery stenosis. The median age and weight of patients were 12 years (range 4 - 15 years) and 24 kg (range 20 - 51 kg), respectively. The CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon inflated successively to expand the stent to desired diameter.

RESULTSTotally 6 stent placement procedures were performed and 8 CP stents (8-zig, 22 - 39 mm in length) were implanted in these 5 patients. All stents but one in a case of right pulmonary artery stenosis were immediately successfully placed in the target lesions without displacement during the procedures. For this case, a repeat procedure was performed and a second CP stent was reimplanted successfully 11 months later. After the procedure, the systolic pressure gradient across the stenosis decreased from (43.43 +/- 25.61) mm Hg (1 mm Hg = 0.133 kPa) to (3.29 +/- 3.09) mm Hg (t = 4.320, P < 0.01) and the narrowest diameter of the stenotic vessels increased from (6.86 +/- 2.04) mm to (13.44 +/- 4.02) mm (t = -4.508, P < 0.01). The percentage of pulmonary artery flow to the ipsilateral lung increased from 11.0% and 13.0% to 47.5% and 52.2% after the procedure in 2 cases of unilateral pulmonary artery branch stenosis, respectively. The ratio of right ventricular to aortic systolic pressure decreased from 62.3% and 72.2% to 27.0% and 33.3% in 2 cases of bilateral branch pulmonary artery stenosis, respectively. Upper limb blood pressure of one case of native CoA dropped greatly from 206/133 mm Hg to 156/95 mm Hg. During a median follow-up of 20 months (range 13 - 34 months), the results have been stable without complications except 2 stents which developed intrastent restenosis 6 months after the procedure.

CONCLUSIONOur experience suggests that the CP stent implantation is safe and feasible for the treatment of vessel stenosis associated with CHD in children and adolescents. The immediate-, early- and interim results are encouraging, but long-term results remain to be further evaluated and demand many more cases to be studied.

Adolescent ; Aortic Coarctation ; therapy ; Cardiac Catheterization ; Child ; Child, Preschool ; Constriction, Pathologic ; Female ; Heart Defects, Congenital ; complications ; therapy ; Humans ; Male ; Platinum ; Pulmonary Valve Stenosis ; complications ; therapy ; Stents ; Treatment Outcome

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.
Arterial Switch Operation ; Arteries* ; Double Outlet Right Ventricle* ; Heart Diseases ; Heart Septal Defects, Ventricular* ; Heart Ventricles ; Humans ; Postoperative Complications ; Pulmonary Valve Stenosis*

BACKGROUND: In the background of increasing silver, the measurement of risk factors of cardiovascular system and abilities of controlling that of the aged are becoming the interest. 'Old age' is one of the risk factors of postoperative cardiovascular complication. We summarized diseases, discovered when randomly examined by transthoracic echocardiography (TTE) and frequencies of the diseases in old aged, older than 60 years. So we could discuss the clinical impact of preoperative TTE at resting state over 60 year old for postoperative cardiovascular system of noncardiac surgery. METHODS: For the period 15months, from September 1, 1999 to January 10, 2001, we examined patients who are older than 60 years, to be scheduled to undergo operation of noncardiac surgery, by using SONOS 1000, made by Hewlett-Packkard, USA. 933 patients (595 men and 337 women) were examined. RESULTS: We examined 993 patients, 595 men (63.8%) and 337 women (36.2%), who were aged from 60 years to 93 years (average 68.5+/-7.1 years old). 87.2% of patients had left ventricular relaxation abnormatilties, average ejection fraction at resting state was 62.0+/-6.8%, which is in normal range and left ventricular end diastolic diameter was 49.2+/-5.4 mm. Becoming older, average left ventricular ejection fraction is decreasing, average of desceleration time and IVRT, parameters of LV relaxation abnormality, are increasing but they are not stastically meaningful.There were four cases of dilated cardiomyopathy (0.4%) in which two cases were incidentally discovered by TTE and they had no specific past histories. 38 persons (4.1%) had pulmonary hypertension (RVSP, higher than 35 mmHg) and they had no specific complication at the operations and postoperative state. In 3 of the patients who had pulmonary hypertension, one was incidentally discovered, had Atrial septal defect and all of three had normal LV function. In our study there were 333 patients (35.7%) of Mitral regurgitation and 343 patients (36.8%) of aortic insufficiency which are pathologic change of aging, and patients severe than grade III were 3 patients (0.3%). One was treated by postoperative congestive heart failure at intensive care unit and other 2 patients could not be followed because of transfer and against discharge. 3 patients was diagnosed by Mitral valve prolapse of patients of Mitral regurgitation (0.3%). In case of Aortic valve stenosis, most popular vavular heart disease in old aged patients, have no symptom unless that is severe, often incidentally discover at TTE in our study at pre-operative state. There were 10 patients of aortic valve stenosis (1.1% frequency), 9 cases were mild ones and remaining one case was severe and received aortic valve replacement at Chest surgery department. CONCLUSION: The prevalence of cardiovascular disease that incidentally discovered at TTE in resting state is similar with the result of the reference in for the old aged population and the group of patients who had no symptom at resting state, could have risks of cardiovascular disease. We cannot predict all of the postoperative cardiovascular complication and risks with TTE at resting state in old age patients, but it was useful for screening of valvular diseases, congenital cardiac disease, pulmonary hypertension. It is also good for carrying out before stress TTE with exercise or dopamine. It has an important role for making decision of performing other stress tests in patients who have cardiovascular risk factors and grouping that need to have intervention therapy like the coronary angiography or not. Besides monitoring the cardiovascular system at operation and postoperation periods, medication during the preoperation period and surgical treatments when cardiovascular abnormalities found at TTE, cardiologic medical treatments are needed to lower the postoperative complication rates and improve the prognosis.
Aging ; Aortic Valve ; Aortic Valve Stenosis ; Cardiomyopathy, Dilated ; Cardiovascular Abnormalities ; Cardiovascular Diseases ; Cardiovascular System ; Coronary Angiography ; Dopamine ; Echocardiography* ; Exercise Test ; Female ; Heart Diseases ; Heart Failure ; Heart Septal Defects, Atrial ; Humans ; Hypertension, Pulmonary ; Intensive Care Units ; Male ; Mass Screening ; Middle Aged* ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Postoperative Complications ; Prevalence ; Prognosis ; Reference Values ; Relaxation ; Risk Factors ; Silver ; Stroke Volume ; Thorax

OBJECTIVEBranch pulmonary artery stenosis is one of the common congenital heart disease. Stent implantation to relieve branch pulmonary artery stenosis (BPAS) is an alternative to failed surgical or balloon angioplasty. The aim of this study was to explore the indication, methods and complications of using balloon expandable stent placement to treat branch pulmonary artery stenosis, and evaluate the results of stent implantation in the treatment of branch pulmonary artery stenosis.

METHODFrom August 2005 to December 2012, 19 patients underwent an attempt at stent implantation. The median age of those patients was 9.1 years (range 4.0-15.0 years). The median weight was 31.7 kg (range 17.0-60.5 kg); 14/19 patients underwent post surgical repair of tetralogy of Fallot, one patient received post surgical repair of pulmonary atresia with ventricular septal defect, one patient underwent post surgical repair of pulmonary atresia with intact septum, one with native left BPAS, and one was after surgical repair of aortopulmonary window and the other truncus arteriosus. CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon was inflated successively to expand the stent to desired diameter. Statistical analysis was performed with the unpaired Student t test.

RESULTA total of 26 stents were implanted successfully in 19 patients. The systolic gradient across the stenosis fell from a median of (36.0 ± 18.3) to (3.8 ± 3.4) mmHg (P < 0.01, 1 mmHg = 0.133 kPa) and the diameter of the narrowest segment improved from (6.0 ± 1.9) to (11.6 ± 3.1) mm (P < 0.01). The right ventricle to aortic pressure ratio fell from 0.68 to 0.49 (P < 0.01). Complications included the following: two stents were malpositioned in the right ventricular outflow tract and one balloon ruptured when dilated the hole of the stent. No other complications occurred. All patients were followed up for 6 months to 6.0 (2.5 ± 1.8) years. One patient underwent stent re-dilation in order to accommodate somatic growth two years later.

CONCLUSIONBalloon expandable stents are safe and effective in relieving BPAS. Stent implantation should be considered the treatment of choice for most patients with BPAS. Stents placed into growing children will require further dilation to keep up with normal somatic growth. Intermediate and long-term follow up studies have shown excellent results after further dilation over time.

Adolescent ; Angioplasty, Balloon ; methods ; Blood Vessel Prosthesis Implantation ; Child ; Child, Preschool ; Constriction, Pathologic ; surgery ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Male ; Postoperative Complications ; epidemiology ; surgery ; Pulmonary Artery ; pathology ; surgery ; Pulmonary Valve Stenosis ; etiology ; surgery ; Retrospective Studies ; Stents ; Treatment Outcome ; Vascular Surgical Procedures ; methods

PURPOSE: This single-center study aimed to assess the clinical features and surgical approaches and outcomes of complete transposition of the great arteries (TGA). METHODS: TGA patients who had undergone surgical correction at the Kyungpook National University Hospital from January 2000 to December 2010, were retrospectively evaluated for patient characteristics, clinical manifestation, preoperative management, intraoperative findings, postoperative progress, and follow-up status. RESULTS: Twenty-eight patients (17 boys and 11 girls, mean age=10.6+/-21.5 days) were included and were categorized as follows: group I, TGA with intact ventricular septum (n=13); group II, TGA with ventricular septal defect (VSD, n=12); and group III, TGA/VSD with pulmonary stenosis (n=3). Group I underwent the most intensive preoperative management (balloon atrial septostomy and prostaglandin E1 medication). Group II showed the highest incidence of heart failure (P<0.05). Usual and unusual coronary anatomy patterns were observed in 20 (71%) and 8 patients, respectively. Arterial and half-turned truncal switch operations were performed in 25 and 3 patients (Group III), respectively. Postoperative complications included cardiac arrhythmias (8 patients), central nervous system complications (3 patients), acute renal failure (1 patient), infections (3 patients), and cardiac tamponade (1 patient), and no statistically significant difference was observed between the groups. Group II showed the mildest aortic regurgitation on follow-up echocardiograms (P<0.05). One patient underwent reoperation, and 1 died. The overall mortality rate was 4%. CONCLUSION: Our study showed favorable results in all the groups and no significant difference in postoperative complication, reoperation, and mortality among the groups. However, our results were inadequate to evaluate the risk factors for reoperation and mortality owing to the small number of patients and short follow-up duration.
Acute Kidney Injury ; Alprostadil ; Aortic Valve Insufficiency ; Arrhythmias, Cardiac ; Arteries ; Cardiac Tamponade ; Central Nervous System ; Follow-Up Studies ; Heart Failure ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Postoperative Complications ; Pulmonary Valve Stenosis ; Reoperation ; Retrospective Studies ; Risk Factors ; Transposition of Great Vessels ; Ventricular Septum

No abstract available.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Echocardiography, Doppler, Color ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/complications/diagnosis/drug therapy/*microbiology ; Fluorescent Antibody Technique ; Heart Septal Defects, Ventricular/*complications/diagnosis/surgery ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Valve Stenosis/*complications/diagnosis ; Purpura, Schoenlein-Henoch/diagnosis/drug therapy/*etiology ; Risk Factors