Coronary Aneurysm ; Coronary Angiography ; Coronary Vessel Anomalies ; Humans ; Vascular Diseases/congenital*

Adult ; Atherosclerosis ; diagnosis ; Chest Pain ; Coronary Aneurysm ; diagnosis ; Coronary Angiography ; Coronary Artery Disease ; diagnostic imaging ; Coronary Vessel Anomalies ; diagnosis ; Coronary Vessels ; diagnostic imaging ; physiopathology ; Electrocardiography ; Humans ; Male ; Risk Factors ; Troponin I ; metabolism ; Vascular Diseases ; congenital ; diagnosis

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
Adolescent ; Arteriovenous Malformations* ; Fontan Procedure ; Heart Defects, Congenital ; Humans ; Male ; Vascular Diseases

BACKGROUND AND OBJECTIVES: Despite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR). SUBJECTS AND METHODS: From 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD). The PVR in these patients was from 6 to 16 wood units . m2, and/or the systolic pulmonary arterial pressure was more than 2/3 of the systemic arterial pressure. RESULTS: The median age was 16 years (range, 9 months-46 years). The median duration of follow-up was 7.4 years (range, 1.4-11.7 years). According to hemodynamic data and clinical symptoms, the initial management comprised targeted medical therapy in four (18%), complete closure in four (18%), and partial closure in 14 patients (63.6%). In the four patients who had a high PVR and negative vasoreactivity, the PVR decreased and vasoreactivity increased after targeted medical therapy; three of these patients underwent cardiac surgery later. Finally, 11 (50%) received targeted medical therapy and 21 patients (95.4%) underwent cardiac surgery. Complete closure resulted in six patients and partial closure in 17 patients. Mortality was observed in two patients. The other 19 patients (91%) had New York Heart Association functional class I. CONCLUSION: Targeted medical therapy may be effective in reducing PVR in patients with congenital heart disease and borderline PVD. A stepwise approach may help to achieve improved outcomes in these patients.
Arterial Pressure ; Cardiology ; Follow-Up Studies ; Heart ; Heart Defects, Congenital* ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary ; Mortality ; Retrospective Studies ; Thoracic Surgery ; Vascular Diseases* ; Vascular Resistance ; Vasodilator Agents ; Wood

BACKGROUND: Pincer nail deformity is a transverse overcurvature of the nail. This study aimed to define the anatomical characteristics of pincer nail deformity and to evaluate the surgical outcomes. METHODS: A retrospective review was conducted on 20 cases of pincer nail deformity of the great toe. Thirty subjects without pincer nail deformity or history of trauma of the feet were selected as the control group. Width and height indices were calculated, and interphalangeal angles and base widths of the distal phalanx were measured with radiography. We chose the surgical treatment methods considering perfusion-related factors such as age, diabetes mellitus, kidney disease, and peripheral vascular disease. The zigzag nail bed flap method (n=9) and the inverted T incision method (n=11) were used to repair deformities. The outcomes were evaluated 6 months after surgery. RESULTS: The interphalangeal angle was significantly greater in the preoperative patient group (14.0degrees+/-3.6degrees) than in the control group (7.9degrees+/-3.0degrees) (P<0.05). The postoperative width and height indices were very close to the measurements in the control group, and most patients were satisfied with the outcomes. CONCLUSIONS: We believe that the width and height indices are useful for evaluating the deformity and outcomes of surgical treatments. We used two different surgical methods for the two patient groups with respect to the perfusion-related factors and found that the outcomes were all satisfactory. Consequently, we recommend taking into consideration the circulatory condition of the foot when deciding upon the surgical method for pincer nail deformity.
Congenital Abnormalities* ; Diabetes Mellitus ; Foot ; Humans ; Kidney Diseases ; Nails, Ingrown ; Onychomycosis ; Osteophyte ; Peripheral Vascular Diseases ; Radiography ; Retrospective Studies ; Toes

Adult ; Coronary Vessel Anomalies ; diagnosis ; etiology ; Humans ; Male ; Smoking ; adverse effects ; Vascular Diseases ; congenital ; diagnosis ; etiology

OBJECTIVETo evaluate the efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty (RVP-PBAV) for congenital aortic stenosis (AS).

METHODA total of sixteen children with AS accepted the treatment with RRVP-PBAV. The patients were at ages 6 months to 15 years, their median age was 5.4 years. Their body weight was between 8.5 and 59.0 kg, average (22.3 ± 16.5) kg. The gradient pressure across the aortic valve was measured for all the patients and aortic regurgitation was observed. The follow-up time ranged from 1 month to 5.5 years.

RESULTAll patients underwent RVP-PBAV successfully. The ratios of balloon/valve were 0.86 to 1.12. The gradient pressure varied from preoperative Δp = (96 ± 32) mmHg (1 mmHg = 0.133 kPa) to the immediate postoperative ΔP = (41 ± 26) mmHg, (P < 0.05). One case had postoperative restenosis, and 3 cases were complicated with bicuspid aortic valve deformity.

CONCLUSIONThe treatment with RVP-PBAV for congenital aortic stenosis is safe and reliable. Rapid ventricular pacing is a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and may decrease the incidence of aortic insufficiency.

Adolescent ; Aorta ; Aortic Valve ; abnormalities ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; therapy ; Balloon Valvuloplasty ; methods ; Body Weight ; Cardiac Surgical Procedures ; Child ; Child, Preschool ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Valve Diseases ; Heart Ventricles ; Humans ; Infant ; Postoperative Period ; Treatment Outcome ; Vascular Malformations

No abstract available.
Aged ; Coronary Vessel Anomalies/radiography/*ultrasonography ; Female ; Humans ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Ultrasonography, Interventional ; Vascular Diseases/*congenital/radiography/ultrasonography

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".
Aneurysm ; Aorta ; Aortic Aneurysm ; Aortic Aneurysm, Thoracic ; Aortic Coarctation ; Aortic Diseases ; Aortic Valve ; Aortic Valve Insufficiency ; Arteries ; Bicuspid ; Coronary Vessels ; Cysts ; Dilatation ; Elasticity ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Valve Diseases ; Humans ; Hypertrophy, Left Ventricular ; Hypoplastic Left Heart Syndrome ; Polymethacrylic Acids ; Rupture ; Tetralogy of Fallot ; Truncus Arteriosus, Persistent ; Vascular Stiffness ; Ventricular Function, Left

Dural arteriovenous fistula (DAVF) is classically defined as abnormal arteriovenous connections located within the dural leaflets. Though the exact etiology is still not clear, they are generally accepted as acquired lesions. However, some DAVFs formed as the congenital disorders are called dural arteriovenous malformations and these lesions with a marked cortical venous reflux are considered to be aggressive and warrant an early intervention. The authors describe a case of 35-year-old man presented with unique type of DAVF. The fistula was located adjacent to the confluence of venous sinuses with multiple feeders. The feeders drained into a large venous pouch just anterior to the confluence which had a bilateral venous drainage. This was associated with multiple cerebellar venous ectasia along the draining cortical vein. It was managed by staged endovascular procedures and complete cure could be achieved. The pathogenesis and technique of embolization of this complex fistula/malformation are also discussed.
Arteriovenous Malformations ; Central Nervous System Vascular Malformations ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Dilatation, Pathologic ; Drainage ; Early Intervention (Education) ; Endovascular Procedures ; Fistula ; Varicose Veins ; Veins