A rare case of an unruptured sinus of Valsalva aneurysm (SVA) in a 2-month-old male Maltese terrier weighing 1.0 kg with a heart murmur is presented. A right SVA and a ventricular septal defect (VSD) were diagnosed by echocardiography and cardiac catheterization. The dog died due to a worsening of his condition. The necropsy revealed the sinus of Valsalva to have a diameter of 7 mm and a VSD hole was on the opposite surface. This report is the first to describe an unruptured SVA in the right coronary cusp of a small dog.
Aneurysm ; Animals ; Cardiac Catheterization ; Cardiac Catheters ; Dogs ; Echocardiography ; Heart Defects, Congenital ; Heart Murmurs ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Male ; Sinus of Valsalva

A unicuspid aortic valve is a rare congenital malformation that frequently presents with valvular dysfunction and dilatation or aortic aneurysm, requiring combined aortic valve surgery and aortic repair. Some patients show severe valve calcification extending into the interventricular septum, possibly resulting in damage to the conduction system during debridement for valve replacement. We present a rare case of severe aortic stenosis with a unicommissural unicuspid aortic valve diagnosed by preoperative transesophageal echocardiography in a 36-year-old man. After composite graft replacement of the aortic valve, aortic root, and ascending aorta, a permanent pacemaker was placed because of postoperative complete heart block.
Adult ; Aorta ; Aortic Aneurysm ; Aortic Valve Stenosis ; Aortic Valve ; Debridement ; Dilatation ; Echocardiography, Transesophageal ; Heart Block ; Heart Defects, Congenital ; Humans ; Transplants

We report a rare case of tricuspid valve and pulmonary valve endocarditis associated with a double-chambered right ventricle in an adult female with pulmonary artery aneurysm and septic pulmonary embolism by Streptococcus mitis. She was treated with aggressive antibiotic therapy followed by debridement of the infective lesion of tricuspid valve, pulmonary valve replacement using xenograft and resection of obstructing muscular bundles in right ventricle.
Adult ; Aneurysm ; Debridement ; Endocarditis* ; Endocarditis, Bacterial ; Female ; Heart Defects, Congenital ; Heart Ventricles* ; Heterografts ; Humans ; Pulmonary Artery ; Pulmonary Embolism ; Pulmonary Valve* ; Streptococcus mitis ; Tricuspid Valve

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.
Abdomen ; Aneurysm ; Congenital Abnormalities ; Dilatation ; Heart ; Heart Atria ; Heterotaxy Syndrome* ; Humans ; Levocardia* ; Liver ; Lung ; Male ; Situs Inversus ; Splenic Artery ; Thorax ; Tomography, Spiral Computed ; Vena Cava, Inferior ; Young Adult

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".
Aneurysm ; Aorta ; Aortic Aneurysm ; Aortic Aneurysm, Thoracic ; Aortic Coarctation ; Aortic Diseases ; Aortic Valve ; Aortic Valve Insufficiency ; Arteries ; Bicuspid ; Coronary Vessels ; Cysts ; Dilatation ; Elasticity ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Valve Diseases ; Humans ; Hypertrophy, Left Ventricular ; Hypoplastic Left Heart Syndrome ; Polymethacrylic Acids ; Rupture ; Tetralogy of Fallot ; Truncus Arteriosus, Persistent ; Vascular Stiffness ; Ventricular Function, Left

Right atrial aneurysm is an extremity rare congenital heart defect. We report a case which was diagnosed during the early fetal stage and operated on in a 2 week old neonate. Following median sternotomy, aneurysmectomy was performed under a cardiopulmonary bypass. Histopathological examination of the resected atiral tissue showed a lipotomatous degeneration and reduction of the muscular elements. There were no postoperative complications.
Aneurysm ; Cardiopulmonary Bypass ; Extremities ; Heart Aneurysm ; Heart Atria ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Postoperative Complications ; Sternotomy

Echocardiography ; Female ; Heart Aneurysm ; congenital ; diagnostic imaging ; Humans ; Infant ; Male ; Ventricular Septum

Adult ; Heart Aneurysm ; congenital ; Heart Atria ; pathology ; Humans ; Male

PURPOSE: Aortic dissection (AoD) is one of the most common catastrophes involving the aorta. Nevertheless, early diagnosis remains to be a challenge in the Emergency Department (ED), particularly in young individuals. In this study, we attempted to identify the characteristics of acute AoD among young individuals, particular in patients with Marfan syndrome. MATERIALS AND METHODS: This was an retrospective chart-review study conducted in a tertiary referring hospital. The hospital database was queried for the combination of AoD and patients under age of 40 years. The medical charts were reviewed to obtain demographic data, clinical data and laboratory characteristics by using a standardized data collection sheet. A comparison between Marfan syndrome and non-Marfan syndrome patients was performed. RESULTS: During the 10-years period, 18 of 344 patients with acute AoD were younger than 40 years-old. Patients with Marfan syndrome developed acute AoD at a younger age than patients without Marfan syndrome. The mean diastolic blood pressure was significantly lower in patients with Marfan syndrome upon presenting to the ED than those without. Patients with Marfan syndrome had trends toward higher risk of development of type A AoD, increased recurrence rate and higher mortality rate than those without. However, statistical significance was not present. CONCLUSION: ED physicians should have high alert to acute AoD in young patients presenting with severe unexplained chest and back pain, particularly in those patients with a history of heart diseases, hypertension, and Marfan syndrome or featuring Marfanoid habitus. Acute coronary syndrome, unexplained abdominal symptoms, and sudden cardiac arrest could be the initial manifestation of AoD in young patients. A low threshold to perform enhanced computed tomography may facilitate early diagnosis and timely treatment in this patient population.
Adult ; Aneurysm, Dissecting/*complications/epidemiology/*pathology ; Aortic Aneurysm/complications/epidemiology/*pathology ; Asian Continental Ancestry Group ; Female ; Heart Defects, Congenital/complications/physiopathology ; Humans ; Hypertension/complications/physiopathology ; Male ; Marfan Syndrome/*complications/*physiopathology ; Middle Aged ; Retrospective Studies ; Young Adult

PURPOSE: Aneurysms of sinus valsalva are rare anormalies thought to be primarily congenital in origin, progressing into death by acute heart failure in cases of rupture. Surgical correction is the only method of treatment. With these clinical implications, we reviewed the clinical characteristics and surgical results of patients with ruptured sinus of valsalva aneurysm. METHODS: Between January 1991 and February 2004, 17 patients with ruptured sinus of valsalva aneurysm were retrospectively reviewed for their clinical symptoms, physical findings, past history, coexistent cardiac anormalies, surgical results, and mid-term prognosis. RESULTS: The 17 patients included 13 men and four women, with a mean age of 30 years(10-59 years). Preoperatively accompanying cardiac anormalies were ventricular septal defect(VSD, eight cases of doubly committed juxta-arterial VSD) and aortic insufficiency(11 cases). During operations, patterns of fistulous tracts were found to be right colonary sinus-right ventricle in 13 patients, right coronary sinus-right atrium in one, noncoronary sinus-right ventricle in two, noncoronary sinus-right atrium in one, and VSD was noticed in 14 patients(all were doubly committed juxta-arterial in type). The defects were closed with a patch in 13 patients, without a patch in four, with concommitant aortic valve replacement in four and with aortic valvuloplasty in two. There were no mortalities during operations or the mid-term follow-up periods(40+/-49 months). CONCLUSION: Because, at least in Orientals, VSD(especially doubly committed juxta-arterial) was accompanied in large numbers of patients with aneurysms of sinus valsalva, preoperative evaluations of this congenital heart disease should be made very careful. And we may need to revise the algorithm of treatment policy in small sized doubly committed juxta-arterial VSD.
Aneurysm* ; Aortic Valve ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Failure ; Humans ; Male ; Mortality ; Prognosis ; Retrospective Studies ; Rupture ; Sinus of Valsalva*