Speciality
Spotlight

 




 


Cardiology


 


   





Congenital Heart Disease

         

  • Zamora R, Rao PS, Llyod TR, et al [ Univ of Arizona, Tucson; St. Louis Univ ; Univ of Michigan, Ann Arbor]

    Intermediate-Term Results of Phase 1 Food and Drug Administration Trials of Buttoned Device Occlusion of Secundum Atrial Septal Defects

    J Am Coll Cardiol 31: 674-676, 1998

         

    Only short-term follow-up studies have investigated the buttoned device used for transcatheter occlusion of secundum atrial septal defects, and the device has been shown to be safe, feasible, and effective. The intermediate-term results of the multi-institutional trial of the button device were evaluated.

        

    Forty-six patients who had successful implantation of the device were prospectively studied and evaluated at 1,6, 12 months and yearly thereafter. Follow-up lasted from 51 to 68 months, with the mean of 60.8 months. The patients were evaluated for history, physical examination, chest radiograph, electrocardiogram, and Doppler echocardiography. The patients ranged in age from 1 to 62 years [mean age 4 years] and in weight from 10 to 105 kgms [mean weight 18 kgms].

        

    The patients had stretched atrial septal defects in which the sizes were 14 mms [plus/minus 4 mms] left to right shunts and 10 mms [plus/minus 3 mms] right to left shunts. Effective occlusion had occurred in 45 of the 46 patients [ 98%], and complete closure in 34 patients [74%]. There was an incidence of residual shunts of 65% at 1 month after implantation, which decreased to 27% at follow-up; all of these were described as trivial. Reintervention for significant residual defects occurred in only 2 patients [4%]; there were no incidents of endocarditis or thromboembolism in the entire follow-up.

         

    In 98% of patients who had the atrial septal device inserted, it provided effective closure for upto 5.5 years; during follow-up the incidence of residual shunts decreased, and no instances of endocarditis or thromboembolism were observed.

  • Kuehl KS, Loffredo CA, Ferencz C (Children’s Natl Med Ctr, Washington, DC; Univ of Maryland, Baltimore)

    Failure to Diagnose Congenital Heart Disease in Infancy

    Pediatrics 103: 743-747, 1999



    Physiologic changes in the circulation after birth mask signs of some congenital cardiovascular malformations (CCVMs) in early infancy. Factors that predict failure to diagnose congenital heart disease in newborns were investigated.



    Data were obtained in 4390 infants with CCVMs between 1981 and 1989. Eight hundred died before the age of 1 year, 76 died before diagnosis of heart disease. Amongst the characteristics of these 76 infants were low birth weight, low gestational age, intrauterine growth retardation and chromosomal abnormalities.



    Common lesions present were coarctation of the aorta, Ebstein’s anomaly, atrial septal defect and truncus arteriorus.



    Diagnosis of CCVMs in infants requires close observation after birth.

  • Ainsworth SB, Wyllie, Wren C (South Cleveland Hosp, Middesbrough, England; Freeman Hosp, Newcastle upon Tyne, England)

    Prevalence and Clinical Significance of Cardiac Murmurs in Neonates

    Arch Dis Child Fetal Neonatal Ed 80: F43-F45, 1999



    Over a period of 2 years, 7204 neonates who did not require intensive care underwent clinical examination. Echocardiography was done in those with clinical heart murmurs. The group was followed up for one year. Of 7204 neonates, 46 (0.6%) had heart murmurs. Of these 46, 13 were found to have normal hearts, 8 had structurally normal hearts but physiological reasons for murmur (patent ductus arterorus), and 25 had heart malformations without any symptoms. During one year of follow-up 32 infants were seen with congenital heart malformations; none of these had a murmur during the initial clinical examination.



    Thus clinical examination identified only 25 of 57 (44%) cardiac malformations seen during the first one year and absence of murmur at birth does not exclude the presence of congenital heart disease.

  • Gregory J, Emslie A, Willie J, et al (Newcastle City Health NHS, England; South Cleveland Hosp, Middesbrough, England; Freeman Hosp, Newcastle upon Tyne, England)

    Examination for Cardiac Malformations at Six Weeks of Age

    Arch Dis Child Fetal Neonatal Ed 80: F46-F48, 1999



    Most infants are examined for cardiovascular malformations when they are 6 to 8 weeks old. The efficacy of this practice was examined in 7132 infants. Murmurs were found in 47; of these 22 were not referred and remained well. The remaining 25 were referred and 14 had normal echocardiograms but 11 had congenital heart disease. Of the remaining with no murmurs, 6 were found to have cardiac defects later.



    Thus routine clinical examination at 6 to 8 weeks does help in detection of congenital heart disease early. 

       
  • Reddy
    VM, McElhinney DB, Sagrado T, et al (Univ of California, San Francisco)

    Results of 102 Cases of Complete Repair of Congenital Heart Defects in Patients Weighing 700 to 2500 Grams

    J Thorac Cardiovasc Surg 117: 324-331, 1999 

       


    Low birth weight (< 2500 g) is a risk factor for poor outcome in corrective surgery for many congenital heart defects. Mere supportive therapy is often associated with severe morbidity.

         


    During a 7-year period, 102 low birth weight infants, with a correctable congenital heart defect underwent complete repair at a median age of 16 days. The most common defects were ventricular septal defect, tetralogy of Fallot and transposition complexes.

         


    There were 10 (10%) early deaths. Cardiac failure caused 4 deaths; there were 1 case each of multiorgan failure, arrhythmia, sepsis, idiopathic coronary intimal necrosis, foot gangrene, and pulmonary hemorrhage. 

         


    Surviving infants were hospitalized for a median of 19 days and received mechanical ventilation for a median of 5 days. Complete follow-up (median 30 months) was available for 88 of 90 early survivors.

         


    Eight late deaths occurred and 8 others underwent 10 interventions. No survivors had clinical evidence of neurological abnormalities.

         


    Thus even complex heart defects can be successfully corrected when surgery is performed early in low birth weight infants. There is also evidence that such infants experience better growth after early rather than later repair.

       
  • Editorial – 

    Homocysteine : a novel risk factor for coronary heart disease in UK Indian Asians.

    Heart 2001; 86: 121-122



    Coronary Heart Disease has higher mortality in UK, Indian Asians than in Whites. 



    Recent studies show that plasma homocysteine concentrations are higher in UK Indian Asians than in European whites. The possibility that homocysteine is a risk factor for coronary heart disease is very real. Homocysteine levels can be reduced by supplementation with vitamin B, especially folic acid. A large scale interventionist study is recommended.    

                  
  • Pigula FA, Khalil PN, Mayer JE, et al (Boston Children’s Hosp)

    Repair of Tetralogy of Fallot in Neonates and Young Infants

    Circulation 100: 11-157S – 11-161S, 1999

                           

    It is controversial whether 1 or 2 stage correction of Tetralogy of Fallot (TOF) is better. Experience at Boston Children’s Hospital with 1 stage repair of TOF was reviewed. 

                             

    Complete repair of TOF with pulmonary stenosis (PS; n=73) and TOF with pulmonary atreria (PA; n=26) was performed in 99 infants (44 girls) aged 2 to 87 days, between January 1988 to December 1996. 

                                   

    There were 3 (3%) in-hospital and 5 (5%) late deaths. Actuarial survival rates at 1 and 5 years were 94% and 91.6% respectively. Smaller body surface and PA were independent risk factors for death. Eight infants had stenal closure delayed 4 to 16 days after correction. Three diaphragmatic plications were performed for paralyzed diaphragms and respiratory impairment 30 to 64 days after surgery. Twenty-nine infants had postoperative arrhythmias, 22 required cardiac catheterization and pulmonary artery or homograft conduit dilatation or stenting and 22 needed 25 reoperations between 1 month and 70 months. Overall freedom from reoperation at 1, 2, and 5 years was 90%, 83%, and 72% respectively.

                                         

    Thus early TOF repair carries an acceptably low mortality but efforts are needed to find ways to limit the need for
    reoperation.   

     




 

 

Speciality Spotlight

 

   

Congenital Heart Disease
         

  • Zamora R, Rao PS, Llyod TR, et al [ Univ of Arizona, Tucson; St. Louis Univ ; Univ of Michigan, Ann Arbor]
    Intermediate-Term Results of Phase 1 Food and Drug Administration Trials of Buttoned Device Occlusion of Secundum Atrial Septal Defects
    J Am Coll Cardiol 31: 674-676, 1998
         
    Only short-term follow-up studies have investigated the buttoned device used for transcatheter occlusion of secundum atrial septal defects, and the device has been shown to be safe, feasible, and effective. The intermediate-term results of the multi-institutional trial of the button device were evaluated.
        
    Forty-six patients who had successful implantation of the device were prospectively studied and evaluated at 1,6, 12 months and yearly thereafter. Follow-up lasted from 51 to 68 months, with the mean of 60.8 months. The patients were evaluated for history, physical examination, chest radiograph, electrocardiogram, and Doppler echocardiography. The patients ranged in age from 1 to 62 years [mean age 4 years] and in weight from 10 to 105 kgms [mean weight 18 kgms].
        
    The patients had stretched atrial septal defects in which the sizes were 14 mms [plus/minus 4 mms] left to right shunts and 10 mms [plus/minus 3 mms] right to left shunts. Effective occlusion had occurred in 45 of the 46 patients [ 98%], and complete closure in 34 patients [74%]. There was an incidence of residual shunts of 65% at 1 month after implantation, which decreased to 27% at follow-up; all of these were described as trivial. Reintervention for significant residual defects occurred in only 2 patients [4%]; there were no incidents of endocarditis or thromboembolism in the entire follow-up.
         
    In 98% of patients who had the atrial septal device inserted, it provided effective closure for upto 5.5 years; during follow-up the incidence of residual shunts decreased, and no instances of endocarditis or thromboembolism were observed.
  • Kuehl KS, Loffredo CA, Ferencz C (Children’s Natl Med Ctr, Washington, DC; Univ of Maryland, Baltimore)
    Failure to Diagnose Congenital Heart Disease in Infancy
    Pediatrics 103: 743-747, 1999

    Physiologic changes in the circulation after birth mask signs of some congenital cardiovascular malformations (CCVMs) in early infancy. Factors that predict failure to diagnose congenital heart disease in newborns were investigated.

    Data were obtained in 4390 infants with CCVMs between 1981 and 1989. Eight hundred died before the age of 1 year, 76 died before diagnosis of heart disease. Amongst the characteristics of these 76 infants were low birth weight, low gestational age, intrauterine growth retardation and chromosomal abnormalities.

    Common lesions present were coarctation of the aorta, Ebstein’s anomaly, atrial septal defect and truncus arteriorus.

    Diagnosis of CCVMs in infants requires close observation after birth.
  • Ainsworth SB, Wyllie, Wren C (South Cleveland Hosp, Middesbrough, England; Freeman Hosp, Newcastle upon Tyne, England)
    Prevalence and Clinical Significance of Cardiac Murmurs in Neonates
    Arch Dis Child Fetal Neonatal Ed 80: F43-F45, 1999

    Over a period of 2 years, 7204 neonates who did not require intensive care underwent clinical examination. Echocardiography was done in those with clinical heart murmurs. The group was followed up for one year. Of 7204 neonates, 46 (0.6%) had heart murmurs. Of these 46, 13 were found to have normal hearts, 8 had structurally normal hearts but physiological reasons for murmur (patent ductus arterorus), and 25 had heart malformations without any symptoms. During one year of follow-up 32 infants were seen with congenital heart malformations; none of these had a murmur during the initial clinical examination.

    Thus clinical examination identified only 25 of 57 (44%) cardiac malformations seen during the first one year and absence of murmur at birth does not exclude the presence of congenital heart disease.
  • Gregory J, Emslie A, Willie J, et al (Newcastle City Health NHS, England; South Cleveland Hosp, Middesbrough, England; Freeman Hosp, Newcastle upon Tyne, England)
    Examination for Cardiac Malformations at Six Weeks of Age
    Arch Dis Child Fetal Neonatal Ed 80: F46-F48, 1999

    Most infants are examined for cardiovascular malformations when they are 6 to 8 weeks old. The efficacy of this practice was examined in 7132 infants. Murmurs were found in 47; of these 22 were not referred and remained well. The remaining 25 were referred and 14 had normal echocardiograms but 11 had congenital heart disease. Of the remaining with no murmurs, 6 were found to have cardiac defects later.

    Thus routine clinical examination at 6 to 8 weeks does help in detection of congenital heart disease early. 
       
  • Reddy VM, McElhinney DB, Sagrado T, et al (Univ of California, San Francisco)
    Results of 102 Cases of Complete Repair of Congenital Heart Defects in Patients Weighing 700 to 2500 Grams
    J Thorac Cardiovasc Surg 117: 324-331, 1999 
       
    Low birth weight (< 2500 g) is a risk factor for poor outcome in corrective surgery for many congenital heart defects. Mere supportive therapy is often associated with severe morbidity.
         
    During a 7-year period, 102 low birth weight infants, with a correctable congenital heart defect underwent complete repair at a median age of 16 days. The most common defects were ventricular septal defect, tetralogy of Fallot and transposition complexes.
         
    There were 10 (10%) early deaths. Cardiac failure caused 4 deaths; there were 1 case each of multiorgan failure, arrhythmia, sepsis, idiopathic coronary intimal necrosis, foot gangrene, and pulmonary hemorrhage. 
         
    Surviving infants were hospitalized for a median of 19 days and received mechanical ventilation for a median of 5 days. Complete follow-up (median 30 months) was available for 88 of 90 early survivors.
         
    Eight late deaths occurred and 8 others underwent 10 interventions. No survivors had clinical evidence of neurological abnormalities.
         
    Thus even complex heart defects can be successfully corrected when surgery is performed early in low birth weight infants. There is also evidence that such infants experience better growth after early rather than later repair.
       
  • Editorial – 
    Homocysteine : a novel risk factor for coronary heart disease in UK Indian Asians.
    Heart 2001; 86: 121-122

    Coronary Heart Disease has higher mortality in UK, Indian Asians than in Whites. 

    Recent studies show that plasma homocysteine concentrations are higher in UK Indian Asians than in European whites. The possibility that homocysteine is a risk factor for coronary heart disease is very real. Homocysteine levels can be reduced by supplementation with vitamin B, especially folic acid. A large scale interventionist study is recommended.    
                  
  • Pigula FA, Khalil PN, Mayer JE, et al (Boston Children’s Hosp)
    Repair of Tetralogy of Fallot in Neonates and Young Infants
    Circulation 100: 11-157S – 11-161S, 1999
                           
    It is controversial whether 1 or 2 stage correction of Tetralogy of Fallot (TOF) is better. Experience at Boston Children’s Hospital with 1 stage repair of TOF was reviewed. 
                             
    Complete repair of TOF with pulmonary stenosis (PS; n=73) and TOF with pulmonary atreria (PA; n=26) was performed in 99 infants (44 girls) aged 2 to 87 days, between January 1988 to December 1996. 
                                   
    There were 3 (3%) in-hospital and 5 (5%) late deaths. Actuarial survival rates at 1 and 5 years were 94% and 91.6% respectively. Smaller body surface and PA were independent risk factors for death. Eight infants had stenal closure delayed 4 to 16 days after correction. Three diaphragmatic plications were performed for paralyzed diaphragms and respiratory impairment 30 to 64 days after surgery. Twenty-nine infants had postoperative arrhythmias, 22 required cardiac catheterization and pulmonary artery or homograft conduit dilatation or stenting and 22 needed 25 reoperations between 1 month and 70 months. Overall freedom from reoperation at 1, 2, and 5 years was 90%, 83%, and 72% respectively.
                                         
    Thus early TOF repair carries an acceptably low mortality but efforts are needed to find ways to limit the need for reoperation.   
     

 

By |2022-07-20T16:44:31+00:00July 20, 2022|Uncategorized|Comments Off on Congenital Heart Disease

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