Quantitative Evaluation of Right-Ventricular Function in Patients with Tricuspid Regurgitation

BACKGROUND: Tricuspid regurgitation (TR) is a common finding that can be detected with use of Doppler echocardiographic technique in patients with right ventricular dysfunction as well as in normal children, adolescents and adult. It is difficult to evaluate the right ventricular function by conventional method, including echocardiography, especially in case with TR. METHOD: To determine the degree and relationship of right ventricular function and duration of TR, we examined the 41 patients with TR associated with heart disease, group 2 (n=19) with no definitive evidence of right ventricular dysfunction nor significant pulmonary hypertension, and group 3 (n=22) with right ventricular dilatation or wall motion abnormality, or history of longstanding pulmonary hypertension or moderate or severe TR, and compared 59 normal subjects (group 1) with trivial or mild TR without definitive heart disease. Using Doppler echocardiography, duration of TR (TRD) and pulmonary ejection period (ET) is measured, and calculated the TR time interval (RTI) by the difference of TRD and ET divided by ET. RESULT: Pre-ejection period (PEP), ET and systolic time interval (STI, PEP/ET) of right ventricle are significantly prolonged in group 2 and 3 compare to those of group 1 (p<0.001 respectively), TRD is significantly prolonged in group 3 compare to those of group 1 and 2, and isovolumic contraction time (IRT), isovolumic relaxation time (IRT) and RTI are significantly different in each group and prolonged in group 2 and 3. RTI is significantly correlated to STI (r=0.56), ICT (r=0.75) and IRT (r=0.94), but independent to heart rate. CONCLUSION: We conclude that Doppler measurement of RTI (TRD-ET)/ET{=(ICT+IRT)/ET} is a simple and useful new index for the evaluation of RV function including systolic and diastolic function.

The usefulness of the genetic markers at the low-density lipoprotein receptor gene locus for the genetic diagnosis of familial hypercholesterolemia / 대한내과학회지

BACKGROUND: Familial hypercholesterolemia(FH) is an autosomal dominant metabolic disorder caused by the mutation in low density lipoprotein receptor(LDLR) gene. However, direct genetic diagnosis of LDLR gene mutation is not easily available because more than 300 mutations have been described in LDLR gene of FH patients. Therefore indirect genetic diagnosis using the genetic markers can be used to follow the inheritance of defective gene in FH families. The purpose of this study was to evaluate the usefulness of indirect genetic markers for detecting identical-by-descent LDLR gene abnormalities in FH families. METHODS: We examined the allele frequency, heterozygosity, polymorphism information content(PIC) of each genetic markers(D19S394, Taq I, Hinc II, Ava II, ATn, D19S221) in 94 unrelated healthy subjects. The genetic polymorphic haplotypes in 3 FH families were also determined. RESULTS: The heterozygosity and PIC values of RFLP's(Taq I, Hinc II, Ava II) were 0.51/0.344, 0.25/0.223, 0.28/0.233 and microsatellite markers(D19S394, ATn, D19S221) were 0.64/0.558, 0.56/0.455, 0.60/0.475. Hinc II and Ava II were significantly linked(|D|=0.72, p< 0.05). The cumulative PIC values of Taq I+Hinc II, Taq I+Hinc II+ATn, D19S394+ATn were 0.520, 0.814, 0.813, respectively. When applied in the FH pedigree, the genetic diagnosis using only one marker was not available in most cases. However, combination of two or more genetic markers could successfully discriminate the affected and unaffected members in FH families. Among the several combinations of the genetic markers, the combination of D19S394 and ATn was supposed to be the most effective and informative. Because one case of recombination was suspected in D19S221 allele, it was thought to be carefully used for genetic diagnosis of FH. CONCLUSION: We concluded that indirect genetic diagnosis using intragenic or extragenic genetic markers was useful for detecting identical-by-descent LDLR gene abnormalities in FH families and the most effective and informative combination of genetic marker seemed to be D19S394 and ATn.

Clinical Observation on Congenital Heart Disease in Adult

BACKGROUND: With the advance of the techniques of echocardiography and cardiovascular surgery, early detection and successful cardiovascular surgery of congenital heart disease is possible in infant as well as in child. And with the advance of the social insurance, the new case of adult congenital heart disease with mild cardiovascular symptom or frank symptom of the pulmonary hypertension is decreasing. We statistically analyze the new case of adult congenital heart disease. METHOD: 92 patients who were diagnosed to congenital heart disease by echocardiography from January 1993 to June 1998 were studied. 2.25 MHz probe for two-dimensional and Doppler echocardiography and biplane 5 MHz phased-array probe for transesophageal echocardiography(Ultramark-9) were used. RESULT: Among 92 patients, 45 patients(48.9%) were male and 47 patients(51.1%) were female and 6 patients(male : 2, female : 4) had multiple congenital heart disease. 61 patients(66.3%) had no definitive cardiovascular symptom and right bundle branch block was most common electrocardiographic abnormality. 32 cases(32.8%) were atrial septal defect, 21 cases(21.5%) ventricular septal defect, 12 cases(12.3%) patent ductus arteriosus, 8 cases(8.2%) congenial bicuspid aortic valve and so on. Female predominance was noted in ventricular septal defect and endocardial septal defect, while male predominance in bicuspid aortic valve and discrete subaortic stenosis. Atrial septal defect and patent ductus arteriosus were no sex difference. 23 cases(25.0%) were between 20 29, 17 cases(18.5%) between 15-19, and 2 cases(2.2%) over 70 years old. CONCLUSION: Our analysis shows similarity to previous report. With advance of the technique of echocardiography and cardiovascular surgery, a few new case of adult congenital heart disease can be diagnosed hereafter.

A Case of Cardiac Tamponade due to Primary Hypothyroidism

Pericardial effusions occur in approximately 30% of patients with untreated hypothyroidism. The effusions are more common in patients with longstanding, severe disease. But, cardiac tamponade is a very rare complication, because of the elasticity of pericardium and slow progression. We experienced a 61 year old female patient with cardiac tamponade due to primary hypothyroidism. She was treated with pericardiocentensis and thyroid hormone replacement, confirmed by echocardiography,thyroid function test and TRH stimulation test.

A Case of Left Main Coronary Artery Disease Treated with Medication

We report a case of 34-year-old woman who had left main coronary artery disease and treated with medication only. She was presented with severe chest pain. Isolated left main coronary disease was demonstrated on selective left coronary angiography. There was no regional wall motion abnormality on echocardiogram with normal global LV systolic function. We considered the lesion as a varient of Takayasu's arteritis. So with anti-anginal medication, treatment started with steroid therapy. After then there was no chest pain. We performed follow-up coronary angiography after 1 month and 3 month in which revealed improved left main lesion. Since then there was no subjective symptom and Thallium-201 SPECT revealed no significant ischemic finding. We think that this case's underlying disease is may be Takayasu's arteritis which has isolated left main stenosis without systemic involvement and improved with medical treatement only.

Cathrter Aboation of The Posteroseptal Accessory Pathways

BACKGROUND: The ablation of accessory pathways(APs) using radiofrequency(RF) energy has been establihed as a primary modality of treatment for atrioventricular reentranr tachycardia with probrn safety and high rate of success. However, the ablation of posteroseptal(PS) APs had been recognized as being more difficult to ablate than those in other location because of the complex three dimensional anatony of the posterior apace, and multifarious approaches have been proposed. We analyzed electrophysiologic characteristics and results of catheter ablation of 70 consecutive patients, who underwent RF ablation of PS APs with or without booster direct current(DC) shock. METHODS: Teh AP location was confirmed to be in the PS region, ablation was attepmted at the atrial aspect of the tricuspid annulus adjacent to the coronary sinus ostium, within the coronary sinus in couding middle cardiac vein, or underneath the mitral annulus close to the septum using retrograde transaortic approach if deemed necessory. A continuous, unmodulated sine wave radiofr-equency generator was used as the source of energy for ablation. The site was considered optimal for ablation when the electrogram obtained from the ablation catheter had one or more of the following characteristics : (1) short VA intervals with an A : V ratio of

Genetic Polymorphism of a Platelet Glycoprotein IIIa as anInherited Risk Factor for Coronary Artery Disease in Koreans

BACKGROUND: Platelet aggregation and thrombus formation within the coronary artery are major factors in acute coronary syndrome. The platelet glycoproteinIIb/IIIa receptor is a pivotal mediator of platelet aggregation. Recently there have been reports that the genetic polymorphism of GPIIIa is an inherited risk factor for coronary artery thrombosis. This study investigated the relation between the Pl(A) polymorphism and coronary artery disease in Korean patients. METHODS: One hundred patients with acute myocardial infarction and unstable angina were enrolled. Coronary angiogram was performed in eighty-one cases. Genomic DNA from peripheral blood was amplified by polymerase chain reaction. Allele-specific restriction digestion was used to determine the Pl(A) genotype. RESULTS AND CONCLUSIONS: The prevalence of Pl(A2) genotype was zero percent in our study group. All patients had the Pl(A1/A1) genotype. These results suggest that Pl(A) genetic polymorphism of GPIIIa is not an inherited risk factor for coronary artery diseases in Koreans.

Electrophysiologic Characteristics of Successfully Ablated Midseptal Accessory Pathway

BACKGROUND: Catheter ablation using radiofrequency energy has been established as the most important mode of treatment in patients with accessory pathway. However the ablation of midseptal accessory pathways had been recognized as being more difficult to ablate than other located pathway because of the low incidence and the difficult localization of ablation site. This paper describes the electrophysiologic characteristics of successfully ablated midseptal accessory pathway using radiofrequency energy. METHOD: Routine electrophysiologic studies were performed in 13 patients with midseptal accessorypathway. Guided by the recording of VA interval, the ablation catheter was positioned in all patients in an area bounded anteriorly by the tip electrode of the His bundle catheter and posteriorly by the coronary sinus ostium. Local electrograms during orthodromic atrioventricular reentrant tachycardia or right ventricular apical pacing were compared for 13 patients with midseptal accessory pathway and consequent 13 patients with posteroseptal accessory pathway. RESULT: 13 patients with midseptal accessory pathway; eight with constant Wolff-Parfinson-White syndrome, one with intermittent Wolff-Parkinson-White syndrome and four with concealed bypass track underwent attempts at ablation of their pathway using radiofrequency energy. 11 accessory pathways were successfully ablated without complication during the firstsession. A second attempt at ablation was made in two patients with success(one; recurred case, the other one; failed case at the first session). In the surface 12-Lead ECG, all eight patientswith constant Wolff-Pakinsin-White syndrome had not shownen Qrs complex at lead 3. Two patient with midseptal accessory pathway had transient left bundle branch block during orthodromic tachycardia. The VA interval during left bundle branch block was not change compared to that during narrow complex tachycardia in both. In all patients with midseptal accessory pathway, the VA interval in his bundle electrogram were almost similar to that in the coronary sinus ostial electrogram, which was not observed in the patients with posteroseptal accessory pathway. CONCLUSION: We suggest that VA interval during orthodromic tachycardia and right ventricular apcial pacing is the most reliable market for identifying midseptal accessory pathway, especially distinguishing from posteroseptal accessory pathway.

The Clinical Presentation and Course of Intramural Hematoma of Aorta

Aortic intramural hematma(IMH) has been known as a variant of acute aortic dissection without intimal rupture. The clinical presentation mimics that of acute aortic dissection. IMH may progress to frank aortic dissection or aortic rupture. Therefore IMH maybe regarded as early sign of developing classic aortic dissection or a precipitating facter. there are Important two questions, The first is whether IMH truly represent a different pathology or simphy the precursor of the conventtional aortic dissection. The second is what the optimal mode of management of IMH is. In this study, To answer these questions, We retrospectively performed this study. Fifteen patients of IMH were included. We could follow 12 patients. Among them extention of IMH to type III aortic dissection has been observed in 2 cases(1 type A and 1 type B). One patients of type A underwent aortic graft stent deployment successfully. In the other patient of type B, who had a history of myocardial infarction and longstanding heart failure by that time, dissection developed at abdominal aorta with renal arterial involvement. The patient died of multiorgan failure despite intensive conservative managements. The remaining ten patients are alive with only medical care and with good clinical outcome. In conclusion we feel that conservative treatment of patients with IMH result in favorable outcome relatively even in the cases involving the ascending aorta. But more longterm follow-up of larger number of patients will provide better guidelines regarding the proper management of IMH.