• Journal of Chest Surgery
• /
• v.12 no.3
• /
• pp.289-296
• /
• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Veterinary Clinics
• /
• v.31 no.4
• /
• pp.298-302
• /
• 2014

An 8-year-old castrated male domestic shorthair cat (Case 1) and 3-year-old castrated male Siamese cat (Case 2) was presented with acute paresis of the hindlimbs, constant open-mouth breathing, and hemoptysis. Heart murmur (Case 1) and gallop sound (Case 2) was ausculated on the left heart base. Radiographs revealed alveolar infiltration of the caudodorsal lung lobes with aerophagea in Case 1 and prominent cardiomegaly in Case 2. Marked concentric hypertrophy of the ventricular septum and free wall, and left atrial enlargement was detected through echocardiography in both cats. Based on the examinations including echocardiography, those cats were diagnosed as hypertropic cardiomyopathy. Abdominal ultrasound revealed echogenic material in the aortic trifurcation region, aortic thromboembolism (ATE). Although prognosis of those animals was guarded, interventional therapeutic approach through direct endovascular thrombolytic therapy was attempted. ATE was visualized through angiography; however dissolving the embolus using interventional thrombolytic approach was not successful due to the extensive thrombus.

• Neonatal Medicine
• /
• v.25 no.3
• /
• pp.126-130
• /
• 2018

Parkes Weber syndrome is a rare congenital vascular anomaly, related to the RAS p21 protein activator 1 (RASA1) gene. It is characterized by capillary cutaneous malformations, bony and soft tissue hyperplasia, and multiple arteriovenous fistulas throughout the affected upper or lower extremity. These arteriovenous fistulas can be associated with life-threatening complications such as bleeding, thrombosis, and high output heart failure. In this report, we present a neonate who had a disproportionately hypertrophied left upper limb with port-wine stain, dystrophy of the left humerus, and hypertrophy of the left clavicle on X-ray, and arteriovenous malformation and massive dilatation of the left subclavian artery on magnetic resonance angiography. Exome sequencing analysis revealed a novel heterozygous splicing mutation (c.1776+2T>A) in the RASA1 gene. To the best of our knowledge, this report is the first case of RASA1-related Parkes Weber syndrome in Korea.

• Journal of Chest Surgery
• /
• v.19 no.2
• /
• pp.265-272
• /
• 1986

We operated on 199 patients of VSD from 1976 to April l986. Among them, patients of VSD whose medical records were available were analyzed clinically. Operation on patients of VSD occupied 23.9% of total open heart surgery [832 cases] during those days. Of the 164 patients, 93 patients were male [56.7%]. 71 patients were female [43.3%]. Their age ranged from 6 months to 28 years and the mean age was 9.5 year and 82.2% of the patients were between 2 and 15 year of age. Of the patients, body weight below 10Kg were 19 cases. The most common complaints were frequent URI and DOE. On Kirklin`s anatomical classification, type II defect was most common [60.1%], type I [38.4%], combined type I+II, type III, combined type II+Ill and combined type II+IV in orders. Associated anomaly was found in 66 patients [42.5%>]. Pulmonary stenosis was most commonly associated cardiac anomaly [8.4%] and aortic insufficiency [7.1%], ASD, Lt. SVC and PDA in orders. There were extracardiac anomalies such as polydactyly, cleft palate, hypospadia and congenital aniridia, etc. Relationship between ventricular hypertrophy and defect size and cardiac cath. data was analyzed. The overall mortality was 7.0% [14 cases] and complication rate was 22.5% [35 cases].

• Journal of Chest Surgery
• /
• v.9 no.2
• /
• pp.198-206
• /
• 1976

A Total of Forty eight patients underwent open-heart surgery for correction of tetralogy of Fallot at the Seoul National University Hospital from January 1974 to October 1976, with an overall survival rate of 77 per cent. Operative mortality varied according to severity of the lesion, age of the patient, nature of previous surgical treatment and presence or absence of an outflow tract patch across the pulmonary valve ring. Eleven patients died in the early postoperative period and thirty seven patients were discharged from the hospital alive. A patch of the right ventricular outflow tract and pulmonary annulus was required to relieve pulmonic stenosis in 24 patients. There were 10 deaths in this group (42%) as compared to 1 death in the group of 24 patients who were corrected without a patch. Operative mortality was especially higher when an inlay patch was placed across the pulmonary valve ring. This may be related to the possibly greater anatomic severity of these cases and to the longer operating time when a patch was used. The electrocardiogram showed right ventricular hypertrophy in 35 cyanotic patients. Intraventricular conduction was normal in 34 patients before operation. It was normal postoperatively in only 5 of 34 patients in this group who survived surgery. Complete right bundle branch block appeared at operation in 21 patients, and 8 patients developed incomplete right bundle branch block. Major causes of death were progressive cardiac failure (4), Complete atrioventricular dissociation (3), bleeding (2), cardiac tamponade (1), and sudden cardiac arrest (1)

• Journal of Chest Surgery
• /
• v.18 no.2
• /
• pp.299-304
• /
• 1985

The partial A-V canal defect consist of ostium primum type atrial septal defect with a cleft mitral anterior leaflet. The clinical findings depend upon the site and size of the left-to-right shunt, the degree of A-V valvular regurgitation, and the degree of resultant pulmonary artery hypertension. We experienced 3 cases of similar condition. The data were as follow: 1. Chest P-A showed increased pulmonary vascularity and moderate cardiomegaly with left atrial enlargement. 2. E.K.G. showed left axis deviation, left atrial enlargement, and left ventricular hypertrophy. 3. Right heart catheterization showed significant 02 step up of SVC-RA and left-to-right shunt. 4. Left ventriculogram showed mitral regurgitation and filling of both atrium. Operative findings were as follow: 1. Primum type atrial septal defect [2x2 cm]. 2. Cleft in the anterior leaflet of the mitral vave. 3. No evidence of ventricular septal defect and tricuspid anomaly. Through a right atriotomy with moderate hypothermia, the mitral cleft was approximated with interrupted sutures. The interatrial communication was closed by a patch of Dacron/pericardium. The patch was attached to junction of the mitral and tricuspid valves along the crest of the ventricular septum using interrupted sutures and the other site using continuous sutures. Postoperative course was uneventful and discharged in good general condition except postoperative bleeding in case 3.

• Journal of Veterinary Clinics
• /
• v.33 no.4
• /
• pp.221-224
• /
• 2016

An 8 month-old male Maltese (weighing 2.0 kg) was referred with loud heart murmur at routine physical exam in local animal clinic. Electrocardiogram found left ventricular hypertrophy pattern (4.5 mV R-wave). Diagnostic imaging studies revealed the elongation of left ventricle (LV) with classic triple bumps on the main pulmonary artery, aorta and left atrium on the ventrodorsal view of radiograph. Echocardiography revealed patent ductus arteriosus (PDA) duct and continuous turbulent shunt flow (maximal velocity 4.83 m/s) between the aorta and pulmonary artery with left to right direction. The PDA in this dog was successfully closed through femoral vein (transvenous approach) using a 5 mm Amplatz$^{(R)}$ Canine Duct Occluder. To the best of author's knowledge, this is the first case of PDA occlusion treated with Amplatz Canine Duct Occluder through femoral vein.

• Korean Journal of Veterinary Research
• /
• v.44 no.1
• /
• pp.125-129
• /
• 2004

A 2-year-old, female, American cocker spaniel dog presented for a 1-year history of severe ascites, exercise intolerance, tachypnea. At that time, she was in an emergency state. First, the dog was stabilized with oxygen therapy. A diagnosis of cardiac problem was made from history, auscultation, radiograph, ECG, and echocardiography. Jugular pulsation was palpated and a harsh, systolic murmur of tricuspid regurgitation was prominent at the right cardiac apex. Tricuspid valve dysplasia (TVD) was confirmed with echocardiography, accompanying enormous myocardial hypertrophy. The clinical signs had been improved for 8 months with careful therapy and periodic abdominocentesis, and ascites was well controlled. The situation, however, became worse quickly in a week because the client did not follow our management schedule. Finally, she died due to dyspnea and shock. After the spontaneous death, necropsy and histopathological examination were performed and when we opened the thorax, a significantly large heart was observed. On histopathological findings, grossly myocardium appeared pale initially, then progressed to yellow and white. Microscopically, there was an extensive hemorrhage along with loss of myocardial striations. Interstitial fibrosis and various degenerative alterations in myocytes were also present.

• Fisheries and Aquatic Sciences
• /
• v.10 no.2
• /
• pp.60-67
• /
• 2007

Myostatin (MSTN; also known as GDF8) is a member of the transforming growth factor ${\beta}-superfamily$ of proteins. MSTN negatively regulates mammalian skeletal muscle growth and development by inhibiting myoblast proliferation. Mice and cattle possessing mutant MSTN alleles display a 'double muscling' phenotype characterized by extreme skeletal muscle hypertrophy and/or hyperplasia. We isolated the full-length cDNA of a novel MSTN gene from S. schlegeli muscle tissue and examined its expression pattern in various tissues. The full-length gene (GenBank DQ423474) consists of 1941bp with an open reading frame of 1134 bp, encoding 377 amino acids that show 62-92% amino acid similarity to other vertebrate MSTNs. The predicted protein contains a conserved proteolytic cleavage site (RXRR) and nine conserved cysteine residues at the C terminus. RT-PCR revealed that the unprocessed and prodomain myostatin mRNAs were predominantly present in muscle, with limited expression in other tissues. However, the mature myostatin mRNA was highly expressed in brain and muscle, intermediately expressed in the gills, intestine, heart, and kidney, and weakly expressed in the liver and spleen.

• Korean Journal of Veterinary Pathology
• /
• v.1 no.2
• /
• pp.125-134
• /
• 1997

Porcine Reproductive and Respiratory Syndrome Virus infection (PRRSV) was confirmed by serology histopathology immunohistochemistry and bacteriologic examination in young pigs. Four suckling and six weaned piglets submitted from three different farms showed coughing sneezing labored rapid abdominal respiration lethargy and anorexia. Grossly apical and cardiac lung lobes appeared mottled with pale to dark tan discoloration. Submandibular and bronchial lymph nodes were tan and enlarged. All piglets were seropositive for PRRSV antibodies by the indirect immunofluorescent antibody(IFA) test. Microscopically lung lesions were characterized by hyperplasia and hypertrophy of type 2 pneumocytes infiltration of mononuclear cells in alveolar intersitium accumulation of necrotic debris in alveolar spaces accompanied by proliferation of alveolar multinucleated syncytial cells. Using immunohistochemical technique PRRSV antigens were demonstrated in alveolar macrophages and type 2 pneumocytes in histologic lung tissue sections. Also PRRSV antigens were detected in brain lymph nodes spleen and heart. Additionally piglets showed nonsuppurative meningoencephalitis mandibular necrotic lymphadenopathy splenic atrophy and myocardial necrosis.