Stress induced cardiomyopathy (SC) is characterized by transient left ventricular (LV) dysfunction in the absence of coronary artery disease. We report on a patient with panhypopituitarism who developed SC resulting from withdrawal of hormonal replacement therapy (HRT). A 52-year-old male visited our hospital for progressively worsening dyspnea. The patient had discontinued HRT 7 days ago, which had been administered for 18 months after transsphenoidal adenomectomy for pituitary macroadenoma. Initial electrocardiogram showed marked sinus bradycardia. Transthoracic echocardiography showed apical ballooning with an LV ejection fraction of 25%. No significant obstructive lesions were observed on coronary angiography. With a clinical diagnosis of SC associated with panhypopituitarism, HRT was restarted, including glucocorticoid and thyroxine, along with standard heart failure management. His LV function had normalized at 2-month follow-up. He remains asymptomatic and administration of beta-blocker and angiotensin converting enzyme inhibitor were discontinued He currently only requires HRT.
Operations for cardiac valvular disease has been progressed in various ways. Since 1949 when Lord Russel operated mitral stenosis by closed technique at Johns Hopkins Hospital then much progress has been achieved and that nowadays severely diseased cardiac valve has been replaced by prosthetic valve, which is almost ideal in hemodynamic aspect, but still it has many problems such as thromboembolism, destruction of red blood cell, pressure gradient, and disturbance of left ventricular function, so in case of delicate situations, valve replacement should be decided carefully. Besides prosthetic valve, there are some kinds of reconstructive procedures and these have been resulted in better prognosis than prosthetic valve replacement in selected cases. So, authors have reviewed 61 Cases of cardiac patients who have been operated reconstructive valvular surgery by cardiopulmonary bypass, at Yonsei University, from Jan. 1963 to Mar. 1976. Out of 61 cases, 9 patients were replaced by prosthetic valve and rest of the patients were operated upon in various reconstructive procedures such as commissurotomy, valvotomy, valvuloplasty, and annuloplasty. Twenty cases of congenital heart diseases with valvular lesion, which had been operated for valvular lesion were also included in this statistics. Out of 9 cases of prosthetic valvular replacement five cases of prosthetic valvular replacement was done combined with other reconstructive procedures after attempted valvuloplasty. Comparative prognosis of both procedures are somewhat variable by reporters, average 19% of mortality after reconstructive surgery and 38% of mortality after prosthetic valve replacement in long term results. Most common cause of death in postoperative period was low output syndrome in both cases. It seems that good preoperative evaluation and proper reconstructive surgery will afford good prognosis in selected cardiac valvular diseased patient.
Ischemic postconditioning (IPost) could decrease ischemia-reperfusion (IR) injury. It has not yet reported whether IPost is useful when ischemic heart disease is accompanied with co-morbidities like hyperthyroidism. The aim of this study was to examine the effect of IPost on myocardial IR injury in hyperthyroid male rats. Hyperthyroidism was induced with administration of thyroxine in drinking water (12 mg/L) over a period of 21 days. After thoracotomy, the hearts of control and hyperthyroid rats were perfused in the Langendorff apparatus and subjected to 30 minutes global ischemia, followed by 120 minutes reperfusion; IPost, intermittent early reperfusion, was induced instantly following ischemia. In control rats, IPost significantly improved the left ventricular developed pressure (LVDP) and ${\pm}dp/dt$ during reperfusion (p<0.05); however it had no effect in hyperthyroid rats. In addition, hyperthyroidism significantly increased basal $NO_x$ (nitrate+nitrite) content in serum ($125.5{\pm}5.4{\mu}mol/L$ vs. $102.8{\pm}3.7{\mu}mol/L$; p<0.05) and heart ($34.9{\pm}4.1{\mu}mol/L$ vs. $19.9{\pm}1.94{\mu}mol/L$; p<0.05). In hyperthyroid groups, heart $NO_x$ concentration significantly increased after IR and IPost, whereas in the control groups, heart $NO_x$ were significantly higher after IR and lower after IPost (p<0.05). IPost reduced infarct size (p<0.05) only in control groups. In hyperthyroid group subjected to IPost, aminoguanidine, an inducible nitric oxide (NO) inhibitor, significantly reduced both the infarct size and heart $NO_x$ concentrations. In conclusion, unlike normal rats, IPost cycles following reperfusion does not provide cardioprotection against IR injury in hyperthyroid rats; an effect that may be due to NO overproduction because it is restored by iNOS inhibition.
SK-1080 is one of the newly developed orally active nonpeptide angiotensinII $AT_1-receptor$ antagonist that selectively acts at $AT_1$ receptor with high affinity. The cardiac effect on ischemia/reperfusion injury of SK-1080 was compared with those of losartan, a prototype of this class, in isolated rat hearts. Isolated perfused rat heart was pretreated with drug for 10 min and then subjected to global ischemia for 30 min followed by reperfusion with- or without drug for 30 min. The possible additive effect of SK-1080 on the platelet aggregation and coagulation in human blood was also studied. We investigated whether SK-1080 effects the platelet aggregation induced by ADP, a platelet agonist partially dependent on $thromboxaneA_2$. The clotting times in the prothrombin time (PT) and activated partial thromboplastin time (APTT) were also examined in human plasma in vitro as coagulation screening test. SK-1080 improved reperfusion function (LVDP, left ventricular developed pressure; PRP, rate-pressure product) in a dose-dependent manner. SK-1080 reduced ADP-induced platelet aggregation compared with vehicle but less than losartan, and did not affect clotting times.
Coronary artery disease is on the rise over the world. Myocardial perfusion SPECT is a well established technique to detect coronary artery disease and to assess left ventricular function. In addition, it has the unique ability to predict the prognosis of the patients. Moreover, the application of ECC-gated images provided the quantitatve data and improved the accuracy. This approach has been proved to be cost-effective and suitable for the emerging economies as well as developed countries. However, the utilization of nuclear cardiology procedures vary widely considering the different countries and region of the world. Korea exits 2-3 times less utilization than Japan, and 20 times than the United States. Recently, with the emerging of new technology, namely cardiac CT, cardiac MR and stress echocardiography, the clinical usefulness of nuclear cardiology has been called in question and its role has been redefined. For the proper promotion of nuclear cardiology, special educations should be conducted since the nuclear cardiology has the contact points between nuclear medicine and cardiology. Several innovations are in horizon which will impact the diagnostic accuracy as well as imaging time and cost savings. Development of new tracers, gamma camera technology and hybrid systems will open the new avenue in cardiac imaging. The future of nuclear cardiology based on molecular imaging is very exciting. The newly defined biologic targets involving atherosclerosis and vascular vulnerability will allow the answers for the key clinical questions. Hybrid techniques including SPECT/CT indicate the direction in which clinical nuclear cardiology may be headed in the immediate future. To what extent nuclear cardiology will be passively absorbed by other modalities, or will actively incorporate other modalities, is up to the present and next generation of nuclear cardiologists.
Lee, Min Young;Lee, Sang Bae;Cha, Hyun Seo;You, Ji Hong;Choi, Eui-Young;Park, Jong Suk
Journal of Yeungnam Medical Science
/
v.34
no.2
/
pp.285-289
/
2017
Pheochromocytoma can present with various symptoms including cardiogenic shock and cardiac arrest. Particularly, in cases of cardiogenic shock of unknown origin, pheochromocytoma should be considered. A 20-year-old woman without any medical history visited our emergency department due to nausea, vomiting, headache, and chest pain. Echocardiography revealed severe left ventricular dysfunction. Mechanical ventilation and veno-arterial extracorporeal membrane oxygenation (ECMO) were implemented owing to her unstable vital signs. For unstable vital sign and cardiogenic shock in a young woman without any previous medical history, pheochromocytoma was considered and diagnosed based on elevated levels of catecholamine derivatives in a 24-hour urine sample. Cardiac function recovered and ECMO was discontinued on the 5th day of hospitalization. She later underwent an elective adrenalectomy and no recurrence was found during the follow-up period. We reported a case of pheochromocytoma which was presented with cardiogenic shock in a young woman with no concomitant disease, and successfully treated with ECMO followed by an elective adrenalectomy.
Kim, Hae-Won;Noh, Kyung-Min;Park, Mi-Young;Lee, Hee-Ran;Lee, Eun-Hee
The Korean Journal of Physiology and Pharmacology
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v.3
no.2
/
pp.223-230
/
1999
Alterations of cardiovascular function associated with various thyroid states have been studied. In hyperthyroidism left ventricular contractility and relaxation velocity were increased, whereas these parameters were decreased in hypothyroidism. The mechanisms for these changes have been suggested to include alterations in the expression and/or activity levels of various proteins; ${\alpha}-myosin$ heavy chain, ${\beta}-myosin$ heavy chain, ${\beta}-receptors,$ the guanine nucleotide-binding regulatory protein, and the sarcolemmal $Ca^{2+}-ATPase.$ All these cellular alterations may be associated with changes in the intracellular $Ca^{2+}$ concentration. The most important regulator of intracellular $Ca^{2+}$ concentration is the sarcoplasmic reticulum (SR), which serves as a $Ca^{2+}$ sink during relaxation and as a $Ca^{2+}$ source during contraction. The $Ca^{2+}-ATPase$ and phospholamban are the most important proteins in the SR membrane for muscle relaxation. The dephosphorylated phospholamban inhibits the SR $Ca^{2+}-ATPase$ through a direct interaction, and phosphorylation of phospholamban relieves the inhibition. In the present study, quantitative changes of $Ca^{2+}-ATPase$ and phospholamban expression and the functional consequences of these changes in various thyroid states were investigated. The effects of thyroid hormones on (1) SR $Ca^{2+}$ uptake, (2) phosphorylation levels of phospholamban, (3) SR $Ca^{2+}-ATPase$ and phospholamban protein levels, (4) phospholamban mRNA levels were examined. Our findings indicate that hyperthyroidism is associated with increases in $Ca^{2+}-ATPase$ and decreases in phospholamban levels whereas opposite changes in these proteins occur in hypothyroidism.
Background: For AVR using conventional prosthetic valves in adult patients with a narrow aortic root, aortic root enlargement is necessary to reduce postoperative pressure gradient across the aortic valve (ΔP). An evaluation of early and mid-term results of aortic root enlargement with AVR and echocardiographic follow up of ΔP and left ventricular function was performed. Method: From Aug. 1991 to Feb. 1998, eighteen patients aged 17 to 59 years(mean, 38$\pm$12 years) underwent Manouguian procedure with AVR. Aortic annular circumstance was enlarged 10.0mm to 18.0mm(mean, 12.6$\pm$6.3mm). Eight patients(44.0%) had NYHA class III status before operation, and seven cases of them underwent concomitant MVR. Valve pathology was ASr in 6 cases, AS in 4 cases, nd ASr+MSr in 8 cases. Replaced valve size was 21mm in 8 cases and 23 mm in 10 cases, and St. Jude Medical mechanical valve was used in 10 cases and Carbomedics in 8 cases. Result: Follow-up duration was 6 to 57 months (mean, 26$\pm$18 months), and total follow-up was 287 patient-year. There were one hospital death and one late death, therefore, actuarial survival rate was 85.7% at 56 months. Peak ΔP wad decreased significantly at postoperative mid-term period as 13$\pm$5mmHg, compared with thepreoperative one (42$\pm$8mmHg) (p<0.01). LVM(gm/$m^2$) was also diminished as 35.8%(115$\pm$36gm/$m^2$)at postoperative mid-term period, compared with preoperative one (179$\pm$56gm/$m^2$)(p<0.05). Conclusion: There were no specific complications related to the procedure. And we could have adequate enlargement of aortic annulus to suitable prosthetic valve that have no effect of patient-prosthese mismatch.
Kim, Ja Hye;Cho, Ja Hyang;Choi, Jin-Ho;Lee, Beom Hee;Yoo, Han-Wook
Journal of The Korean Society of Inherited Metabolic disease
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v.14
no.1
/
pp.37-41
/
2014
Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.
Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.
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