Idiopathic infantile arterial calcification: case report.

Idiopathic infantile arterial calcificationI (IIAC) is a rare disorder characterized by calcium deposition in the internal elastica lamina of medium and large arteries and it has been defined in term of molecular genetics. It is usually fatal, approximately 85% of all patients die within the first months of life owing to ischemia of vital organs. Death from myocardial infarction usually occurs in the first 6 months. Calcification in a peripheral artery with electrocardiogram (ECG) changes of occlusive coronary artery disease suggests the diagnosis. It is caused by mutations in the ENPP1 gene localized on chromosome 6q22 and has OMIM number 208000. A case with fatal evolution at the end of the first month is presented.

Nosocomial infections in pediatric cardiac surgery, Italy.

OBJECTIVE: To evaluate the incidence of nosocomial infection (NI) in pediatric patients who received cardiothoracic surgery and to identify possible associated risk factors. DESIGN: Prospective observational study. SETTING: The cardiac surgery and cardiac intensive care units at the Regina Margherita Children's Hospital, Turin, Italy. PATIENTS: All patients who underwent surgery from July 20, 1998, to July 19, 1999, were enrolled, except patients with operative catheterization only. METHODS: Clinical data were collected daily from July 20, 1998, to July 19, 1999. NIs were diagnosed according to US Centers for Disease Control and Prevention criteria. RESULTS: 104 patients were included in the present study, 80 (76.9%) of whom underwent extracorporeal circulation. The NI ratio was 48.1% (50/104); the percentage of patients with NI was 30.8% (32/104): 23.1% developed one infection, 7.7% two or more. The rate of NI was 2.17 per 100 days of hospitalization (50/2,304). The most common pathogen was Pseudomonas aeruginosa. Important risk factors were length of preoperative admission >5 days, total length of admission >10 days, open chest during postoperative phase, and cyanotic heart disease. There was a significant association between sepsis and central venous catheterization for 3 days or more. Rate of sepsis was 19 per 1,000 catheter days (16/852). CONCLUSION: NIs represent a frequent complication for children who undergo heart surgery. Based on our data, we suggest decreasing the preoperative stay as much as possible. The higher NI incidence in patients with an open chest postoperatively suggests that an alternative antibiotic strategy should be considered for these patients.

Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, Ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An Ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.

Surgical retrieval of an embolized central venous catheter in a premature baby.

Embolization of central venous catheter fragments is usually treated with percutaneous interventional techniques, which are difficult to apply in infants with very low birth weight. We surgically removed a catheter fragment in a preterm neonate, to avoid the impending thrombosis of the right pulmonary artery. The operation was performed with a nerve hook introduced through a tiny incision in the vessel's wall. The procedure was well tolerated, and no stricture remains at the site of incision.

Arteriovenous fistulas of the placenta in a singleton fetus with large atrial septal defect and anomalous connection of the umbilical veins.

Arteriovenous fistulas of the placenta rarely occur in singleton pregnancies. This report describes the fetal and neonatal hemodynamic pattern in a singleton gestation in which multiple placental artery-to-vein anastomoses were associated with a large atrial septal defect and a single umbilical artery with an anomalous connection of the persistent right and left umbilical veins. Possible links between the extracardiac vascular malformation and the congenital heart defect are discussed.

[Endoscopic palliation of dysphagia due to anastomotic recurrences after esophageal surgery and total gastrectomy due to carcinoma]. / La palliazione endoscopica della disfagia secondaria a recidiva dell'area anastomotica dopo resezione esofagea e gastrectomia totale per carcinoma.

BACKGROUND: Dysphagia is the main symptom of locally recurrent esophageal and gastric carcinoma and generally suggests extensive disease. The safety and efficacy of endoscopic palliation in patients who developed locally recurrent disease after surgery is evaluated. METHODS: Twenty-six males and 12 females, with an average age of 63.5 years were included in the patients group. In 12 patients there was an esophago-gastric anastomosis, in 26 an esophago-jejunostomy, after total gastrectomy. Anastomotic recurrences were divided into predominantly polypoid (16 cases) and predominantly stenosing (22 cases). Three treatment modalities were employed: endoscopic dilation (6 cases), Nd-Yag laser therapy (16 cases) and prostheses (16 cases). RESULTS: Successful endoscopic treatment was obtained in 33 out of 38 patients (86.8%). Major complications occurred in 5 patients (15%) with a mortality rate of 6%. CONCLUSIONS: The endoscopic palliation is effective. Technically, endoscopic treatment is easier to perform, with better results, when dealing with polypoid rather than stenosing recurrences.

Berry syndrome, a complex aortopulmonary malformation: one-stage repair in a neonate.

Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.

[Results of endoscopic Nd-Yag laser treatment of inoperable colorectal carcinoma]. / Risultati del trattamento perendoscopico con Nd-YAG laser del carcinoma del colon-retto non operabile.

Between November 1986 and December 1993, 94 patients underwent endoscopic laser therapy for inoperable colo-rectal cancers. According to the main clinical symptoms, tumors were divided into obstructing and bleeding; A Nd-YAG non contact laser was used at power setting of 70 to 100 watt. In 28 cases laser therapy was carried out after electrorection of the tumor and in 22 cases after dilation; in 41 cases it was associated with radiation therapy. In 90% of bleeding tumors and in 70% of obstructing tumors we achieved a good result in the short term. In 50% of bleeding tumors and in 65% of obstructing tumors the symptoms recurred after an interval of 8.2 and 6.1 weeks. The main survival period was 11.2 months for bleeding tumors and 7.4 months for obstructing tumors. Two patients with a small rectal cancer are free to neoplasia at 19 and 26 months after laser therapy.

Self-expanding metal stents for palliation of inoperable carcinoma of the esophagus and gastroesophageal junction.

OBJECTIVES: Rapid palliation of malignant dysphagia is usually possible with endoscopic implantation of plastic prosthesis, but this device has a high rate of complications. Recently expandable metal stents have become available that may have a reduced complications rate. METHODS: This report details our experience with 32 patients treated from September of 1992 through June of 1994. Twenty-three patients were treated primarily with the Ultraflex esophageal prosthesis, and five patients were treated with postoperative malignant stricture, three with failed laser therapy and one with postradiation therapy malignant stricturing. Implantation was successful in 30/32 patients (94%). No major bleeding or perforation followed placement. The dysphagia score improved dramatically from 3 to 0.5. Twenty-six patients had a follow-up of at least 30 days. No stent migration occurred. Food impaction was seen in three patients, tumor ingrowth in three, and overgrowth in one patient. The median survival was 6.2 months with a range of 1.8-11.3 months. CONCLUSIONS: Expandable metal stents are effective and safe for palliation of malignant obstruction of the esophagus and gastro-esophageal junction. However, long term problems remain to be addressed, such as ingrowth by tumor, food impaction, and limitation of stent expansion by tumor rigidity.

Echocardiographic assessment of aortic valve replacement with stentless porcine xenografts.

Stentless porcine xenografts (SPXs) implanted in the aortic position have potential hemodynamic advantages over traditional valve prostheses because of the lack of a rigid stent. Twenty-four patients (mean age 59 years) who underwent aortic valve replacement with SPXs were studied by echocardiography early after and 26 +/- 10 months (range 8 to 40) after operation. Peak and mean gradients, as well as aortic valve area, did not change significantly from baseline (16.3 +/- 8 and 9.8 +/- 5.6 mm Hg, and 1.78 +/- 0.63 cm2, respectively) to follow-up study (12.5 +/- 5 and 7.7 +/- 3 mm Hg, and 1.8 +/- 0.65 cm2, respectively). At baseline, color flow Doppler imaging showed aortic valve regurgitation where the leaflets coapted centrally in 17 of 24 patients (trivial, n = 14; mild, n = 3). Besides the central leak, paravalvular regurgitation was seen in 4 patients (trivial, n = 3; mild, n = 1). At follow-up, 18 of 24 patients had aortic valve regurgitation (trivial, n = 11; mild, n = 6; and moderate, n = 1). New valvular regurgitation (graded as trivial, n = 2; mild, n = 2; and moderate, n = 1) was detected in 5 patients, and new paravalvular regurgitation (graded as mild) developed in 1 patient. Two patients underwent repeat operation for valve-related complications: (1) rupture of a valve cusp with acute pulmonary edema, and (2) fibrotic stenosis of the left coronary ostium with unstable angina. In conclusion, this study demonstrates good hemodynamic performance of the SPX in the aortic position.(ABSTRACT TRUNCATED AT 250 WORDS)

[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. / Studio mediante eco-color Doppler transesofageo del flusso venoso polmonare prima e dopo correzione di vizi valvolari.

AIM OF THE STUDY: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease. METHODS: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID). Paired Student's t-test was used for analysis of data; a p value < 0.05 was considered statistically significant. RESULTS: In mitral stenosis and insufficiency, as well as in pulmonary stenosis, the VmaxS/VmaxD and IS/ID ratios were constantly < 1. Aortic stenosis, on the contrary, showed a normal preoperative pattern of pulmonary venous flow, which did not change after correction. All other successful corrections (17 surgeries, 4 angioplasties) were characterised by an increase of VmaxS/VmaxD and IS/ID ratios. (Mitral stenosis: VmaxS/VmaxD 0.80 +/- 0.31 vs 1.4 +/- 0.5, p = 0.006; IS/ID 0.86 +/- 0.77 vs 1.62 +/- 0.62, p = 0.016. Severe mitral insufficiency: VmaxS/VmaxD -0.71 +/- 0.32 vs 1.19 +/- 0.32, p < 0.0001; IS/ID 0.41 +/- 0.19 vs 1.04 +/- 0.31, p = 0.006. Moderate mitral insufficiency: VmaxS/Vmax D 0.38 +/- 0.04 vs 0.95 +/- 0.06, p = 0.001; IS/ID 0.32 +/- 0.05 vs 0.95 +/- 0.07, p = 0.02. Pulmonary stenosis: VmaxS/VmaxD 0.43 +/- 0.23 vs 1.09 +/- 0.35, n.s. e IS/ID 0.49 +/- 0.34 vs 0.92 +/- 0.65, n.s.). Failure to return to a normal pulmonary venous pattern was observed in the 2 cases of partially successful mitral valvuloplasty (one of which was subsequently transformed into a mitral valve replacement with immediate normalisation of the pattern) and in the 2 cases of incomplete relief of a pulmonary stenosis after pulmonary valvuloplasty. CONCLUSIONS: Though preliminary, these observations suggest a high sensitivity of this method and, therefore, a possible role of pulmonary venous pattern studies in the assessment of the efficacy of treatment in mitral and pulmonary valve disease.

Regurgitant flow of mitral valve prostheses: an intraoperative transesophageal echocardiographic study.

To assess the regurgitant characteristics of mitral biologic and mechanical prostheses immediately after implantation, intraoperative transesophageal echocardiography was performed in 27 patients, aged 32 to 69 years, undergoing open-heart surgery for rheumatic heart disease (n = 19), mitral valve prolapse (n = 3), malfunctioning prostheses (n = 3), or periprosthetic leaks (n = 2). The prostheses included 13 biologic (Carpentier-Edwards) and 14 mechanical valves (five Starr-Edwards, five Medtronic-Hall, and four Bjork-Shiley). Physiologic transvalvular regurgitant flow was detected in both biologic and mechanical prostheses. The spatial extent of the regurgitant jets was usually greater in the mechanical than in the biologic valves, and systolic jets, characteristic of each type of valve, were visualized consistently. Trivial periprosthetic jets (PPJs) were observed in many implanted valves (14/27). The median maximal jet area was 0.46 cm2 (range 0.1 to 1.5 cm2). Cardiopulmonary bypass was reinstituted in two patients. In one patient a PPJ was judged extensive enough (area 3.6 cm2) to warrant surgical revision of the implant, but no dehiscence was found. In the other patient a turbulent PPJ (area 5.5 cm2) was associated with a 0.5 cm dehiscence at the surgical inspection. In conclusion, (1) all mitral prostheses exhibit physiologic transvalvular regurgitation, (2) trivial mitral PPJ is a common finding in newly implanted mitral valves and does not require the revision of the implant, and (3) further experience based on larger series of patients is required to determine the maximal acceptable size of a mitral PPJ detected by intraoperative transesophageal echocardiography.

Intraoperative echocardiography by a new miniaturized epicardial probe: preliminary findings.

Miniaturized probes constitute recent progress in the field of epicardial echocardiography. We recently used a new miniaturized probe, derived from a standard transesophageal probe, in a series of 12 adult patients who underwent cardiac surgery in order to test the possibility of obtaining new views for epicardial imaging. This study demonstrates the feasibility and safety of performing intraoperative echocardiography when using a miniaturized epicardial probe. This probe may be placed on a broader epicardial and vascular area, thus overcoming the size limitations of the commonly used epicardial probes. The major limitation found with the miniaturized probe, however, was the inability to obtain a true four-chamber view from the ventricular apex, due to the difficulty of holding the probe motionless between the apex and the diaphragm while the heart is beating. Although extensive experience with larger groups of patients and different pathologies will be required to define the full potential of this new probe, the advent of the miniaturized probe may further expand the applicability of epicardial echocardiography in pediatric patients during surgery for congenital heart disease.

[Echocardiography-guided endomyocardial biopsy]. / La biopsia endomiocardica ecoguidata.

We have performed 20 echocardiography-guided endomyocardial biopsies in 16 patients, totalling 98 bioptic samples. In each case fluoroscopy was available to supplement the echocardiographic findings. The right ventricle was biopsied in 17 cases, the left ventricle in 3. All 3 left ventricular biopsies and 14 out of 17 right ventricular biopsies were satisfactorily guided by echocardiography. An adequate echocardiographic window was not obtained in 3 cases of right ventricular biopsies and the procedures were carried out under fluoroscopy. In 5 cases (25%), totalling 10 samples, echocardiography showed a somewhat different position of the bioptome from that suggested by fluoroscopy, thus guiding a significant repositioning of it. Finally, in one patient, echocardiography promptly visualized a severe pericardial effusion, due to cardiac perforation, thus allowing its immediate drainage.