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[Transcatheter closure of ductus arteriosus with coils. Immediate results. Initial experience in Mexico].

From March to May 1996 we performed transcatheter closure of patent ductus arteriosus with spring coils in 4 patients. Age ranged from 5 month to 11 years (mean 4.3 years). One of them had two previous Rashkind occluders with persistant shunt and other case had pulmonary valve stenosis, in this case we performed pulmonary valvuloplasty and after that closure of ductus arteriosus. Mean pulmonary arterial pressure was 10 to 38 mmHg (mean 18), QP/QS was 1.2 to 5.0 (mean 3.2). Immediate total occlusion occurred in two cases and the other had trivial shunt by doppler echocardiography. No complications were detected. In conclusion we believe that this device is another no surgical alternative to treat persistant ductus arteriosus. It is effective, it has a low cost, and all the patients are candidates. The procedure carries minimum risk and has no mortality.

Child↗

New triple-lumen balloon catheter for percutaneous (pulmonary) valvuloplasty.

Percutaneous transluminal balloon pulmonary valvuloplasty has been performed since 1982. In publications, the most critical problems mentioned are severe systemic hypotension and bradycardia due to stasis of blood flow. A triple-lumen balloon was developed to allow more rapid inflation and deflation and to preserve the preexistent forward blood flow during maximal inflation. Only a slight systemic hypotension and no bradycardia were seen in the first two patients with pulmonary valve stenosis treated with this type of balloon. Thus, the maximal inflation time is not a critical parameter, whereas with the former types of balloon, maximal inflation times were critical, and, if longer than 5 to 10 sec, severe systemic hypotension was registered. We conclude that the triple-lumen pulmonary valve-dilatation balloon catheter is superior to the single-lumen balloon catheter used up to now.

Angioplasty, Balloon↗

Significance of a cardiac murmur as the sole clinical sign in the newborn.

A cardiac murmur is a very common finding in the first few days of life. It is traditionally believed that lesions creating left-to-right shunts do not present so early. This study was aimed to define and to classify the causes of a murmur in a newborn with an otherwise normal examination. All echocardiograms performed on newborns aged 1-5 days who were referred for evaluation of a murmur in a 3-year period were reviewed. Newborns with additional clinical signs or antenatal diagnosis of a cardiac disease were excluded. Of 20,323 live births, there were 170 newborns referred for echocardiogram solely because of a murmur. Of these, 147 (86%) were found to have structural heart defects. The most common lesions found were those creating left-to-right shunts (66%). Ventricular septal defect was the most common single lesion (54/147, 37%), followed by patent ductus arteriosus (34 newborns, 23%). The combination of both was found in 10 newborns (7%). Six newborns (4%) had pulmonary valve stenosis and three (2%) aortic valve stenosis. Seven newborns (5%) had unforeseen complex heart disease. For five of them, delayed diagnosis would have resulted in potentially life-threatening conditions. There was no correlation between the category of lesion and the age of presentation. The audible threshold of a murmur correlated with a maximum instantaneous gradient of 25 mm Hg (range 11-46 mm Hg). Thirteen percent of newborns with isolated heart murmur had no identifiable structural heart disease. These data suggest that most asymptomatic newborns presenting with a murmur in the first days of life have structural heart disease. Some of the more serious defects would not have been diagnosed without early echocardiography. A left-to-right shunt, particularly a ventricular septal defect, may cause a murmur even the first day of life and is probably more common that has been acknowledged.

Aortic Valve Stenosis↗

[Transcatheter pulmonary valvuloplasty].

We describe the first case of balloon pulmonary valvuloplasty performed in this country. This 12 year old girl had a transvalvular gradient of 94 mmHg and after the procedure it improved to 20 mmHg. Four months later, cardiac catheterization did not show any significative change in pulmonary valvular gradient (26 mmHg). We describe the technique, hemodynamic findings, complications, and advantages of this procedure. We also review the literature. We consider that balloon valvuloplasty should be the first choice procedure in the treatment of patients with congenital pulmonary valve stenosis.

Blood Pressure↗

Continuous wave Doppler assessment of patients subjected to pulmonary balloon valvoplasty.

To evaluate the role of Doppler ultrasound in accurately quantitating patients with valvar pulmonary stenosis, we studied 30 patients (20 patients undergoing balloon pulmonary valvoplasty before and after the procedure, and 10 patients who had previously undergone pulmonary balloon valvoplasty) by Doppler echocardiography and cardiac catheterization. The peak systolic gradient was calculated by the two methods within 24 hours of each other. The maximal velocity of blood flow was obtained by non-imaging, continuous wave Doppler ultrasound examination performed from various parasternal and subcostal positions and the valve gradient was calculated using a modified Bernoulli's equation. Transpulmonary peak systolic gradient calculated by Doppler examination ranged from 9 to 159 mm Hg (mean 54.8 +/- 34.5 mm Hg) and correlated well with values obtained at cardiac catheterization (r = 0.94, P less than 0.001). Thus our study shows that accurate quantification of pulmonary valve stenosis can be reliably undertaken non-invasively utilizing Doppler echocardiography.

Adolescent↗

[Cerebral abscess disclosing congenital heart disease].

Cerebrovascular accidents and brain abscesses are the most worrisome complications of cyanogenic cardiac lesions in infants and young children, but remain rare in the adult. The authors report the case of a brain abscess which led to the subsequent discovery of a congenital cardiac malformation consisting of a ventricular septal defect and moderate pulmonary valve stenosis. Complete correction of the cardiac lesions was carried out three months after neurosurgical treatment (needle biopsy/drainage).

Brain Abscess↗

[Total cavo-pulmonary anastomosis in heart surgery for the correction of univentricular heart].

The total cavopulmonary anastomosis is one of the alternative surgical procedures which can be performed in the management of the most complicated congenital heart diseases. It was the first time in Hungary that this surgical management was performed successfully in a girl with univentricular heart, pulmonary valve stenosis, malposition of the great arteries, who was operated on palliative procedure previously.

Abnormalities, Multiple↗

Aortopulmonary septal defects. A review of the literature and report of ten cases.

From 1981 to 1992, 10 infants with aortopulmonary septal defect (APSD) underwent surgical repair. The mean age at operation was 5.6 +/- 5.5 months, and the mean weight 4.6 +/- 2 kg. Intracardiac associated anomalies were as follows: ventricular septal defects (7 cases), tetralogy of Fallot (2 cases), aortic valve stenosis (2 cases), atrial septal defect (3 cases), patent ductus arteriosus (3 cases), pulmonary valve stenosis (1 case). Cardiac catheterization was performed in 8 out of 10 patients. Eight patients had type I (proximal) defect, 1 had type II (distal) defect and 1 had type III (absent aortopulmonary septation) defect. A variety of surgical procedure was employed. APSD closure with hemoclip was feasible in 3 cases with small window. In 6 patients, during a period of cardiopulmonary by-pass, a side biting clamp was positioned on the ascending aorta close the defect; the border of the window was divided leaving a flap of pulmonary wall on the left side to close the aortic defect; the pulmonary artery was repaired by an autologous pericardial patch. In the patient with type III APSD, aortopulmonary septation was carried out through a transwindow approach. Associated anomalies were repaired in all infants except one. Hospital mortality was 10% (1 case). No late deaths occurred. At a mean follow-up of 47 +/- 35 months 8 patients are asymptomatic and 1 is awaiting for repair of associated anomalies. Conclusions. APSD is a rare but nonetheless well identifiable anomaly. Surgery is indicated as soon as the diagnosis is established, regardless of the patient's age.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗

Accuracy of continuous wave Doppler predicted gradient in patients undergoing balloon pulmonary valvoplasty.

14 patients who underwent balloon valvoplasty had trans-pulmonic gradient evaluated by continuous wave Doppler echocardiography. Maximum systolic gradients measured from Doppler flow velocities were correlated with peak to peak gradient obtained at cardiac catheterisation. Prior to valvoplasty, there was good correlation between the Doppler maximum gradient (92.85 +/- 34.7mm Hg) and the peak to peak catheter gradient (105.57 +/- 56.60 mm Hg), (r = 0.91, p = less than 0.001). Immediately after balloon valvoplasty, the maximum Doppler gradient did not correlate with the peak to peak catheter gradient (r = 0.33, p = NS). Exclusion of patients with infundibular gradients improved the correlation coefficient between the Doppler maximum and peak to peak catheter gradient to 0.69. At late restudy following valvoplasty, when regression of infundibular stenosis was noted in 6 out of 8 patients, the Doppler maximum and catheter peak to peak gradient had excellent correlation (r = 0.97, p = less than 0.001). In patients with lone valvular gradient immediately following valvoplasty and at late restudy, maximum Doppler gradients correlated well with catheter gradients in 14 estimations (r = 0.66, p = less than 0.01). This study shows that the non-invasive quantification of pulmonary valve stenosis can be reliably undertaken, using continuous wave Doppler echocardiography before balloon valvoplasty and during follow-up, after the procedure when the infundibular stenosis has regressed. The presence of an infundibular gradient immediately after balloon dilatation makes the Doppler prediction less reliable.

Adolescent↗

Catheter interventions in adult patients with congenital heart disease.

Adult patients with congenital heart disease provide a wide variety of challenges for the interventional cardiologist. Procedures can broadly be divided into dilatation or closure. The most common interventions in our own practice are closure of atrial septal defects and patent foramen ovale, although closure of postoperative interatrial communications in Mustard, Senning, or Fontan patients is also possible. Transcatheter patent ductus arteriosus closure is also now routine, and occlusion of coronary artery fistulae can be safely attempted using coil embolisation. Balloon dilatation of pulmonary valve stenosis has excellent success rates, and dilatation or stenting of pulmonary artery stenoses is also beneficial in selected cases. Although aortic valve dilatation in adults has mixed results, dilatation or stenting of aortic coarctation is now becoming more widespread, and has promising results. Dilatation procedures may also be applicable to the postoperative patient with conduit or baffle stenosis.

Adult↗

Attenuated pulmonary vascular obstructive disease in double-outlet right ventricle and mitral stenosis.

A young woman with double-outlet right ventricle, mitral stenosis, and high systemic pulmonary artery pressure is described. Pulmonary vascular morphometric analysis indicated considerably less pulmonary vascular obstructive disease than might otherwise have been expected. Total repair was performed and resulted in a marked fall in pulmonary artery pressure. Pulmonary vascular morphometry may be indicated in selected patients before palliative management is selected over complete repair.

Adult↗

Balloon dilatation of critical stenosis of the pulmonary valve in neonates.

Percutaneous balloon dilatation was attempted in 15 consecutive neonates (mean age 7.3 (range 1-27) days and weight 3.2 (range 2.5-4.1) kg) with critical stenosis of the pulmonary valve. Dilatation was successful in 11 (73%) patients. The mean balloon to annulus ratio was 1.1 (range 0.6-1.77). The ratio of right ventricle to femoral artery systolic pressure decreased from a mean (1 SD) of 1.4 (0.32) before to 0.8 (0.24) after dilatation and the transvalvar gradient decreased from 81 (29.7) mm Hg before to 33 (27.7) mm Hg after dilatation. All four (27%) patients in whom dilatation was unsuccessful underwent surgical valvotomy. Complications of balloon dilatation occurred in three (20%) patients; these included retroperitoneal haematoma (one) and iliofemoral venous occlusion (two). In one (7%) patient severe hypoxia and hypotension developed when the valve was crossed with a guide wire and balloon catheter. Despite successful dilatation he died 7 days after the procedure. During a mean (1 SD) follow up of 2 (1.7) years, seven (64%) of the 11 patients remained free of important restenosis. One patient required repeat dilatation three weeks after the initial procedure. In three (27%) patients restenosis developed 4-9 months after dilatation and all three had surgical valvotomy. Of the four patients initially referred for surgery three required a second operation and one required balloon dilatation. Percutaneous balloon dilatation gave effective relief of critical pulmonary stenosis in most neonates but complications and restenosis requiring surgery were common.

Blood Pressure↗

Pulmonary vascular-bronchial interactions: acute reduction in pulmonary blood flow alters lung mechanics.

BACKGROUND: Postoperative pulmonary hypertension in children after congenital heart surgery is a risk factor for death and is associated with severe acute changes in both pulmonary vascular resistance and lung mechanics. OBJECTIVE: To examine the impact of changes in pulmonary blood flow on lung mechanics in preoperative children with congenital heart disease, in order to assess the cause-effect relation of pulmonary vascular-bronchial interactions. DESIGN: Prospective, cross sectional study. SETTING: Cardiac catheterisation laboratory, general anaesthesia with mechanical ventilation. INTERVENTIONS: Variation of pulmonary blood flow (Qp) by either balloon occlusion of an atrial septal defect before interventional closure, or by complete occlusion of the pulmonary artery during balloon pulmonary valvuloplasty for pulmonary valve stenosis. MAIN OUTCOME MEASURES: Ventilatory tidal volume (Vt), dynamic respiratory system compliance (Cdyn), respiratory system resistance (Rrs). RESULTS: 28 occlusions were examined in nine patients with atrial septal defect (median age 9.5 years) and 22 in eight patients with pulmonary stenosis (median age 1.2 years). Normalisation of Qp during balloon occlusion of atrial septal defect caused no significant change in airway pressures and Rrs, but there was a small decrease in Vt (mean (SD): 9.61 (0.85) to 9.52 (0.97) ml/kg; p < 0.05) and Cdyn (0.64 (0.11) to 0.59 (0.10) ml/cm H(2)O*kg; p < 0.01). These changes were more pronounced when there was complete cessation of Qp during balloon valvuloplasty in pulmonary stenosis, with a fall in Vt (9.71 (2.95) to 9.32 (2.84) ml/kg; p < 0.05) and Cdyn (0.72 (0.29) to 0.64 (0.26) ml/cm H(2)O*kg; p < 0.001), and there was also an increase in Rrs (25.1 (1. 7) to 28.8 (1.6) cm H(2)O/litre*s; p < 0.01). All these changes exceeded the variability of the baseline measurements more than threefold. CONCLUSIONS: Acute changes in pulmonary blood flow are associated with simultaneous changes in lung mechanics. While these changes are small they may represent a valid model to explain the pathophysiological impact of spontaneous changes in pulmonary blood flow in clinically more critical situations in children with congenital heart disease.

Adolescent↗

[Corrected transposition of the great vessels associated with severe pulmonary stenosis in a 59-year-old man].

The case of a 59-year-old man with corrected transposition of the great arteries (CTGA) is reported. This anomaly is rare in adult patients, uncommon in patients over 50. Most of the cases with CTGA are diagnosed in childhood because of associated cardiac abnormalities. When isolated or complicated by other mild anomalies, CTGA can cause no symptoms until adult life or can even go unrecognized. However, most of these patients present with congestive heart failure when they are 40 or 50 years old. Our patient was admitted to the hospital because of heart failure. Cardiac catheterization and selective angiography demonstrated a CTGA associated with severe pulmonary valve stenosis. The case here reported shows that CTGA even when associated with other cardiac abnormalities, can be a relatively benign lesion, and can be revealed in adult life by signs of cardiac failure.

Humans↗

Infant methylenetetrahydrofolate reductase 677TT genotype is a risk factor for congenital heart disease.

OBJECTIVE: Recently, an association between the homozygous C677T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene in infants with congenital neural tube defects or congenital oral clefts has been shown. However, no data are available so far with respect to the MTHFR 677TT genotype in children with underlying structural congenital heart disease (CHD). METHODS: We investigated the MTHFR genotype in 114 Caucasian CHD patients aged newborn to 16 years (median 0.6 years; 53% male) and in 228 age- and sex-matched healthy controls. RESULTS: In childhood patients with CHD the homozygous MTHFR 677TT genotype was found in 21 out of 114 subjects (18.4%) compared with 21 out of 228 controls (9.2%; odds ratio (OR) 2.2, 95%-confidence interval (CI) 1.2-4.3; P=0.027). In patients with pulmonary valve stenosis, hypoplastic left heart syndrome, coarctation of the aorta, aortic valve stenosis or subaortic stenosis the frequency of the TT genotype varied between 38 and 67% with corresponding ORs from 6.1 (CI, 1.4-27.5; P=0.034) to 20.4 (CI, 1.8-235.0; P=0.025), whereas in other structural CHD the frequency of this genotype was not significantly different from the controls. CONCLUSIONS: With the present study we can show for the first time that the embryonal MTHFR 677TT genotype is significantly associated with the development of structural congenital heart malformations during early pregnancy. It remains to be clarified, whether this genotype is at least a risk marker or a risk factor for structural congenital heart malformations.

Adolescent↗

Factors determining immediate and medium-term results after pulmonary balloon valvuloplasty.

OBJECTIVE: To determine factors influencing immediate and medium-term results of Pulmonary Balloon Valvuloplasty (PBV) for pulmonary valve stenosis. MATERIAL AND METHOD: Between 1995 and 2001, the authors carried out PBV in 25 consecutive patients. Two treatment outcomes including immediate and medium-term results were analyzed. The immediate results were classified into two groups based on the pulmonary systolic pressure gradient (PG) immediately after dilation: group 1 with a PG < or = 35 mmHg and group II with a PG > 35 mmHg. At medium-term follow-up of 6 to 60 months, echocardiographic evaluations were analyzed and divided into two groups: group A with a PG < or = 25 mmHg and group B with a PG > 25 mmHg. Demographic characteristics and hemodynamic parameters of immediate and medium-term results were compared and analyzed. RESULTS: Immediately after PBV the right ventricular systolic pressure (RVSP) decreased from 115.3 +/- 37.6 mmHg to 67.0 +/- 28.5 mmHg (p < 0.001). The pulmonary systolic pressure gradient decreased from 90.4 +/- 37.9 mmHg to 39.3 +/- 25.6 mmHg (p < 0.001). Nine of the 25 patients (group 11; 36%), with incomplete immediate relief of the obstruction, had more symptoms, a higher baseline PG, higher right atrial pressure, higher RVSP, and a higher systolic pressure ratio. Six of the 25 patients (group B; 24%), with suboptimal medium-term results, had a higher right ventricular voltage on the electrocardiogram, higher pre-dilation PG, and higher RVSP. CONCLUSION: Successful medium-term outcomes following pulmonary balloon valvuloplasty were achieved in 76% of the patients, with a greater success rate (91%) in patients with a lower right ventricular voltage electrocardiogram (R wave amplitude in V1) < or = 21 mm, a prevalvuloplasty systolic gradient < or = 90 mmHg, and right ventricular systolic pressure < or = 125 mmHg.

Adolescent↗