Zohair Al Halees

One- to Ten-Year Follow-Up Results of Balloon Angioplasty of Native Coarctation of the Aorta in Adolescents and Adults

OBJECTIVES We attempted to evaluate the role of balloon angioplasty in the treatment of discrete coarctation of the aorta in adolescents and adults, with special emphasis on long-term results. BACKGROUND Controversy persists over the use of balloon dilation for the treatment of native coarctation of the aorta. METHODS Between July 1986 and January 1997, 43 consecutive adolescent and adult patients with discrete coarctation of the aorta underwent balloon angioplasty. One- to 10-year follow-up data of 37 patients, including results of cardiac catheterization and magnetic resonance imaging (MRI), form the basis of this study. RESULTS No early or late deaths occurred. Balloon angioplasty produced a reduction in the peak to peak coarctation gradient from a mean +/- SD of 69 +/- 24 mm Hg (95% confidence interval [CI] 61 to 76) to 12 +/- 8 mm Hg (95% CI 10 to 14.8) (p < 0.001). Follow-up catheterization 12 months later (37 patients) revealed a residual gradient of 6.7 +/- 6 mm Hg (95% CI 4.6 to 8.9); 3 (7%) of 43 patients had suboptimal results with development of recoarctation, defined as peak gradient >20 mm Hg, with successful repeat angioplasty. A small aneurysm developed at the site of dilation in 3 (7%) of the 43 patients. MRI follow-up data 1 to 10.8 years (mean 5.2 +/- 2.7) after angioplasty (37 patients) revealed no new aneurysm or appreciable change in the size of the preexisting aneurysm in the three patients. The blood pressure had normalized without medication in 27 (73%) of 37 patients at follow-up examination. CONCLUSIONS Balloon angioplasty is safe and effective and should be considered a viable alternative to operation for treatment of discrete coarctation of the aorta in adolescents and adults.

Severe tortuosity and stenosis of the systemic, pulmonary and coronary vessels in 12 patients with similar phenotypic features: a new syndrome?

We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.

Arterial complications following cardiac catheterization in children less than 10 kg.

We sought to determine if a higher dose of heparin would reduce arterial complications in patients weighing 10 kg or less undergoing cardiac catheterization to investigate congenital heart disease. Sixty patients were given either 100 (group A) or 150 (group B) IU·kg−1 of heparin in a double-blinded randomized manner. Initial arterial access was established using a 4F cannula in all patients. Mean activated clotting time measured 20 minutes following heparin administration was significantly lower in group A than in group B (199 versus 251 seconds). Only 3 out of 60 patients (5%) required treatment for loss of femoral pulse. The age, weight, activated clotting time, length of catheterization procedure, time taken to establish arterial access, and the duration of arterial cannulation were comparable between the groups. Weight under 4 kg, age under 1 month, and cannula size larger than 4F were identified as independent risk factors for the development of arterial complications. Arterial access using a 4F cannula is a safe procedure in children weighing 10 kg or less. The incidence of significant arterial complications is low, and they do not appear to be preventable by a higher dose of heparin.

Relevance of apolipoprotein E polymorphism for coronary artery disease in the Saudi population.

BACKGROUND The apolipoprotein E alleles epsilon2 and epsilon4 have been reported as independent risk factors for coronary artery disease (CAD) and as predictors for the development of atherosclerosis. METHODS AND RESULTS We determined by polymerase chain reaction the distribution of apolipoprotein E polymorphism in 320 Saudi blood donors (BD), 96 CAD patients, and 40 control subjects who had undergone angiography. Compared to controls, only epsilon4 was elevated in CAD patients. More than 61% (P <.0001) of the patients had angina, and 52.1% (P <.05) were diabetic; both of these factors were strongly associated with the presence of allele epsilon2. The epsilon2 allele was also associated with hypertension, elevated serum triglycerides, and total cholesterol. On the other hand, the allele epsilon4 appeared to be associated with increased risk of CAD and was also associated with hypertension, 3-vessel disease, and restenosis. CONCLUSIONS Accordingly, epsilon4 may be associated with increased risk of CAD, whereas epsilon2 appears to be a predictor of several risk factors for atherosclerosis.

Repair of moderate aortic valve lesions associated with other pathology: an 11-year follow-up

OBJECTIVES The presence of moderate aortic valve (AV) lesions associated with other pathologies that require surgery presents a problem since ignoring or replacing the valve seems unsatisfactory. AV repair can be an attractive alternative if shown to perform satisfactory. METHODS To evaluate this possibility, all consecutive AV patients who underwent operation between July 1988 and July 1999 were reviewed. Out of 1764 AV patients, 239 (14%) underwent repair and 86 (study group) had moderate lesions associated with mitral (73), tricuspid (33), coronary disease (5) and others (8). Mean age was 28 years (range 2--66); 78% were rheumatic, 71% were in sinus rhythm and 71% in NYHA class III--IV. RESULTS There were seven hospital deaths (8%) and three patients were lost to follow-up (95% complete). Late mortality was 8% and 10-year actuarial survival was 86 +/- 4.5% (excluding hospital mortality). There were four (5%) embolic events (actuarial freedom 94 +/- 3.5%). Twenty-one patients required reoperation with two mortalities. The AV was not touched in five patients. In the remaining 16, the AV was replaced. Only one patient had isolated AV replacement while in all others, additionally, the mitral, tricuspid, or both required surgery. All reoperated patients had rheumatic etiology. Actuarial freedom from AV dysfunction at 8 years was 68 +/- 7.5%. CONCLUSIONS Repair of associated moderate AV lesion is worth considering even in a predominantly young rheumatic population.

Balloon dilation of native aortic coarctation in infancy

Die Ballondilatation zur Behandlung der nativen Aortenisthmusstenose ist für das Neugeborenen- und Säuglingsalter umstritten. ZFK 956 Im Zeitraum von Februar 1985 bis August 1999 wurde am King Faisal Hospital in Riyadh bei 80 Patienten mit nativer Aortenisthmusstenose im Alter unter 12 Monaten eine Ballondilatation durchgeführt. Der systolische Druckgradient konnte dabei von durchschnittlich 45,6±19,4 mmHg auf 17,9±13,8 mmHg gesenkt werden. Bei 55 (68,8%) Patienten verlief die Prozedur initial erfolgreich (Restgradient <20 mmHg). Bei 12,5% der Patienten wurden Intimarisse entdeckt, 21,3% hatten einen passageren, therapierbaren Femoralarterienverschluss. Bei 2 Patienten wurden, direkt nach dem Ersteingriff, Aortenaneurysmen entdeckt, von denen eines operativ entfernt werden musste. Schwere Komplikationen oder Todesfälle in Verbindung zur Ballondilatation traten nicht auf. Langzeitergebnisse konnten bei 66 von 80 (82,5%) Patienten in einem Zeitraum von 6–174 Monate (Median 29 Monate) nach dem ersten Eingriff erhoben werden. Insgesamt wurde bei 22/66 (33%) durchschnittlich 10,9±15,2 Monate nach der ersten Ballonangioplastie eine Redilatation notwendig, davon waren 16 erfolgreich, 4 wurden nach erfolgloser Ballondilatation chirurgisch versorgt. 30 der 66 Patienten, die zu den Nachuntersuchungen kamen (45%) nach der ersten Dilatation keine weitere interventionelle Therapie. Bei der Analyse der Altersgruppen zeigt sich, dass Neugeborene und Säuglinge bis 3 Monate mit 90 und 62% eine signifikant erhöhte Restenoserate aufweisen. Hier kann die Ballondilatation allenfalls als palliative Therapie bei schwerer linksventrikulären Dysfunktion oder anderer Allgemeinerkrankung eingesetzt werden. Balloon dilation of aortic coarctation in neonates and infants remains controversial. Between 2/1985 and 8/1999, 80 patients <12 months of age, with native aortic coarctation underwent balloon dilation. The systolic pressure gradient across the stenosed area was reduced significantly acutely from a mean of 45.6±19.4 mmHg to 17.9±13.8 mmHg. In 55 (68.8%) patients, the procedure was initially successful with a residual gradient of <20 mmHg. In 12.5% of patients, intimal tears were detected after dilation. In 21.3% of patients, obstruction of the femoral artery occurred, which responded to heparin or streptokinase in all. Two patients developed aneuryms immediately after the first intervention. In 1 case, surgery was performed with a successful aneurysmectomy. Severe complications or death in relation to the procedure were not detected. Long-term follow-up was obtained in 66 of 80 (82.5%) patients in a period between 6 to 174 months (median 29 months). In 22/66 (33%) of the infants, within a mean period of 10.9±15.2 months after first intervention, a redilation was necessary. Sixteen of 22 were successful procedures. In the remaining six patients, surgery was performed electively. A total of 30/66 who had follow-up (45%) patients remained free from reintervention or surgery after the first procedure. Further analysis of the data according to age showed that neonates and infants ≤3 months of age had a 90% and 62% higher residual stenosis rate, respectively, than infants >3 months. In this young age group, balloon dilation can only be recommended as palliation in young infants with severe left venticular dysfunction or in the case surgery is prohibitive for other reasons.

Comparison of the effect of left ventricular volume and pressure overload on β-adrenoceptor density in left heart valvular disease

The sympathetic responses in left heart valvular disease may depend on the ventricular load conditions. We proposed to evaluate this possibility by comparing the effects of left ventricular pressure (LVP) and volume (LVV) overload on beta-adrenoceptor density and ligand binding affinity in lymphocytes and in the four myocardial chambers in rheumatic heart valvular patients. Receptor activity was determined by radioligand binding using [125I]iodocyanopindolol. In the lymphocytes (n = 45), the beta-adrenoceptor density was reduced by 88% (P < 0.001) in LVP patients (n = 15) and 79% (P < 0.001) in LVV patients (n = 30) compared with 23 controls. In the myocardium, the receptor density of the LVP (n = 12) was attenuated by 55% (P < 0.05) in the left ventricle, 42% in the right ventricle, 13% in the left atrium, and 37% in the right atrium, while in LVV patients (n = 22) it decreased by 73% (P < 0.01) in the left ventricle, 62% (P < 0.05) in the right ventricle, 30% in the left atrium, and 34% in the right atrium compared with 15 controls. Thus, the reduction in density was greatest in lymphocytes and least in the atria in both groups. The decrease in ventricular density of the LVV group was similar to the reduction in the lymphocytes and two-fold higher than in the atrial density. These alterations were significantly greater for the LVV than for the LVP group. The Kd for the myocardial receptor binding to [125I]iodocyanopindolol was not significantly influenced in either group, but was lower in the lymphocytes. These findings suggest that in patients with left heart valvular disease, there is a significant attenuation in both peripheral and myocardial beta-adrenoceptor density. The decrease in receptor density is significantly greater in the left ventricular volume overload than in the left ventricular pressure overload patients.

Effect of left ventricular pressure and volume overload on α-adrenoceptor activity in patients with rheumatic heart valvular disease

We have investigated the possibility that the various left ventricular load conditions may exert different effects on the sympathetic function by comparing the influence of volume (VOL) and pressure (POL) overload on platelet alpha-adrenoceptor activity, plasma catecholamines and cAMP in 44 patients with rheumatic heart valvular disease. Receptor activity was determined by radioligand binding methods, catecholamines by HPLC using an electrochemical detector, and cAMP by radioimmunoassay. The mean alpha-adrenoceptor density (Bmax) of the control group (n = 29) was 4.71 +/- 0.41 fmol per 10(7) platelets and the corresponding dissociation constant (Kd) was 2.47 +/- 0.15 nM. In VOL patients, the density was elevated by 70% (P < 0.0001), but it remained unchanged in the POL patients. In contrast to the Bmax, the Kd of the VOL group was not changed, and it increased by 34% (P < 0.01) in the POL group. Norepinephrine was elevated by 91% (P < 0.05) in POL, and epinephrine increased by 65% (P < 0.05) in POL and 71% (P < 0.05) in VOL. These results suggest that the sympathetic nervous system responds to left ventricular volume overload by increasing alpha-adrenoceptor density with no apparent change in receptor affinity toward [3H]-yohimbine binding, and to left ventricular pressure overload by decreasing their binding affinity without a parallel decrease in receptor density. The increase in receptor density in VOL is accompanied by an increase in plasma epinephrine, and the decrease in binding affinity in POL is associated with increased plasma norepinephrine and epinephrine levels.

The Choice of Valve Prosthesis: Are the Guidelines for Everyone?

In Asia, as in many parts of the world, a signifi cant number of patients of various ages present with valvular heart disease requiring intervention. Considering the population of Asia, this number will be much greater than in the West. As the population is aging, the proportion with degenerative aortic and mitral valve disease is expected to rise. The prevalence of rheumatic fever remains high in many parts of Asia, ranging from 1.2 to 21 per 1,000, and many patients with valve disease are relatively young. Congenital cardiac defects are common, and these can involve the aortic and mitral valves. The result is patients with a very wide age range and spectrum of valvular heart disease, from the neonate and infant to the very old.

Left ventricular mechanics after arterial switch operation: a speckle-tracking echocardiography study.

Background The arterial switch operation (ASO) is nowadays the standard procedure for the repair of dextro-transposition of the great arteries (d-TGA). Reduced exercise capacity, coronary artery abnormalities, and reversible myocardial perfusion defects have been demonstrated in patients who have undergone ASO. Despite this, indices of systolic function, assessed by standard echocardiography, are within the normal range. Speckle-tracking echocardiography (STE) can detect early subclinical myocardial abnormalities in several diseases even in the presence of normal left ventricular (LV) ejection fraction. Aim To assess LV systolic myocardial deformation and torsion in asymptomatic ASO patients with normal LV ejection fraction (≥55%) by using STE. Methods We studied 62 asymptomatic patients (26 women) who have undergone single-stage ASO for simple d-TGA, aged 8.5 ± 5.7 years, with a normal LV ejection fraction (≥55%); 31 age and sex comparable controls (14 women), aged 7.9 ± 4.9 years. Results In patients who have undergone ASO, global LV longitudinal strain was significantly lower than that in controls (−19.2 ± 2.9% vs. −22.7 ± 2.4%, respectively, P < 0.0001). Longitudinal deformation was significantly impaired in the anterior and both anterior and posterior septal walls. In patients who have undergone ASO global circumferential strain and LV torsion were similar to controls. At multivariate analysis global LV longitudinal strain was significantly correlated only with age at surgery (P = 0.005). Conclusion We demonstrated a significant reduction in longitudinal myocardial deformation correlated with the age at surgical repair, despite a normal LV ejection fraction, in the largest series of asymptomatic ASO patients by using STE. Our findings suggest early (⩽7 days) operation on d-TGA patients and continued monitoring of ventricular function by STE.