Cantu syndrome and lymphoedema.

Three female patients with Cantu syndrome were studied, two of whom were adults presenting with the complication of lymphoedema, as described earlier in a male patient with this syndrome. The aim of this study is to report the clinical characteristics of these three new cases and to emphasize that lymphoedema, as observed in two of the patients described here, has been observed in 11.5% of patients with Cantu syndrome and that heterochromia iridis, observed in one patient, is probably a new feature of this condition.

[Pregnancy and tetralogy of Fallot with and without surgical correction]. / Embarazo y tetralogía de Fallot con y sin corrección quirúrgica.

BACKGROUND: Tetralogy of Fallot is the most common cyanotic congenital cardiac lesions. Although pregnancy in patients with corrected tetralogy of Fallot usually have a satisfactory outcome, there may have maternal cardiovascular complications. OBJECTIVE: To report our experience in monitoring and treatment of 16 pregnancies in 14 women with tetralogy of Fallot. PATIENTS AND METHOD: Prospective study performed in 16 pregnancies of 14 patients with tetralogy of Fallot, who attended the services of Cardiology and High Risk Pregnancy in the Hospital de Gineco-Obstetricia of the Centro Médico de Occidente (Mexico), from January 1997 to January 2010. Nine women had total surgical correction and five hadn't. All patients obtained complete study protocol and tests of fetal wellbeing. RESULTS: Hemoglobin and hematocrit were significantly higher in the group without surgical correction; this group also had lower oxygen saturation and right ventricular enlargement. Of the 16 pregnancies, five were resolved vaginally, the other by cesarean section. The cyanotic mothers had premature termination of pregnancy, lower birth weight and Apgar slightly deteriorated. There were no maternal or neonatal deaths, neither cardiac malformation in newborns. CONCLUSIONS: There are more risks for the binomial in patients with uncorrected tetralogy of Fallot and in those operated with significant residual lesions. A greater anatomical impact was significantly correlated with major hemoglobin and minor oxygen saturation, which are the most important risk factors for adverse fetal outcomes.

Bentall procedure in ascending aortic aneurysm: hospital mortality.

BACKGROUND: Ascending aortic aneurysm disease (AAAD) shows a low frequency, heterogeneous behavior, high risk of rupture, dissection and mortality, making elective surgery necessary. Several procedures have been developed, and the Bentall technique is considered as the reference standard. The objective was to describe the hospital mortality of AAAD surgically treated using the Bentall procedure. METHODS: We carried out a descriptive study. Included were 23 patients with AAAD who were operated on between March 1, 2005 and September 30, 2008 at our hospital. Data were obtained from clinical files, and descriptive statistics were selected for analysis. RESULTS: The study population was comprised of 23 patients with an average age of 46 years; 83% were males. Etiology was nonspecific degeneration of the middle layer with valve implication in 43%, bivalve aorta in 22%, Marfan syndrome, Turner's syndrome and poststenotic aneurysms each represented 9%, and Takayasu disease and ankylosing spondylitis 4% each. Associated heart disease was reported in six (26%) patients as follows: aortic coarctation (2), ischemic cardiopathy (1), atrial septal defect (1), severe mitral insufficiency (1) and subaortic membrane (1). Procedures carried out were Bentall surgery in 20 (87%) patients and aortoplasty with valve prosthesis in three (13%) patients. Complications reported were abnormal bleeding with mediastinal exploration (17%), nosocomial pneumonia (13%), arrhythmia (13%), and septic shock (9%). Mortality was reported in three (13%) patients due to septic shock and ventricular fibrillation. CONCLUSIONS: Surgical mortality with the Bentall procedure is similar to published results by other specialized centers. Events related to the basic aortic pathology, surgical technique, aortic valve prosthesis and left ventricular dysfunction encourage longterm studies with follow-up.

Procedimiento de Bentall en la enfermedad aneurismática de la aorta ascendente: mortalidad hospitalaria / Bentall procedure in ascending aortic aneurysm: hospital mortality

Introducción: La enfermedad aneurismática de la aorta ascendente (EAAA) se caracteriza por su baja frecuencia, comportamiento heterogéneo, riesgo de rotura y disección, que conllevan elevada mortalidad, por lo que la cirugía electiva es fundamental. Se han desarrollado diversos procedimientos quirúrgicos, considerándose la técnica de Bentall el estándar de referencia. Se describe la mortalidad hospitalaria de la EAAA tratada quirúrgicamente mediante el procedimiento de Bentall. Material y métodos: Estudio descriptivo en el que se incluyeron 23 pacientes con EAAA operados entre el 1 de marzo de 2005 y el 30 de septiembre de 2008; la información fue obtenida de los expedientes clínicos. Resultados: Los 23 pacientes correspondieron a 1.2 % de las cirugías efectuadas. Edad media de 46 años (rango 16 a 74), sexo masculino 83 %. Etiología: degeneración inespecífica de la capa media con implicación valvular 43 %, aorta bivalva 22 %, síndrome de Marfán, de Turner y aneurismas posestenóticos, 9 % cada uno. Enfermedad de Takayasu y espondilitis anquilosante, 4 % cada uno. Enfermedad cardiaca asociada en seis (26 %): coartación aórtica (2), cardiopatía isquémica (1), comunicación interauricular (1), insuficiencia mitral severa (1) y rodete subaórtico (1). Procedimientos realizados: cirugía de Bentall 20 (87 %), aortoplastia con prótesis valvular tres (13 %). Complicaciones: sangrado anormal con reintervención 17 %, neumonía nosocomial 13 %, arritmias 13 %, choque séptico 9 %. Mortalidad tres (13 %): choque séptico y fibrilación ventricular. Conclusiones: La mortalidad hospitalaria para la cirugía de Bentall fue semejante a la registrada en otros centros especializados. Los eventos relacionados con la patología aórtica, técnica quirúrgica, prótesis valvular aórtica y la disfunción ventricular izquierda, obligan a realizar estudios de seguimiento a largo plazo.

BACKGROUND: Ascending aortic aneurysm disease (AAAD) shows a low frequency, heterogeneous behavior, high risk of rupture, dissection and mortality, making elective surgery necessary. Several procedures have been developed, and the Bentall technique is considered as the reference standard. The objective was to describe the hospital mortality of AAAD surgically treated using the Bentall procedure. METHODS: We carried out a descriptive study. Included were 23 patients with AAAD who were operated on between March 1, 2005 and September 30, 2008 at our hospital. Data were obtained from clinical files, and descriptive statistics were selected for analysis. RESULTS: The study population was comprised of 23 patients with an average age of 46 years; 83% were males. Etiology was nonspecific degeneration of the middle layer with valve implication in 43%, bivalve aorta in 22%, Marfan syndrome, Turner's syndrome and poststenotic aneurysms each represented 9%, and Takayasu disease and ankylosing spondylitis 4% each. Associated heart disease was reported in six (26%) patients as follows: aortic coarctation (2), ischemic cardiopathy (1), atrial septal defect (1), severe mitral insufficiency (1) and subaortic membrane (1). Procedures carried out were Bentall surgery in 20 (87%) patients and aortoplasty with valve prosthesis in three (13%) patients. Complications reported were abnormal bleeding with mediastinal exploration (17%), nosocomial pneumonia (13%), arrhythmia (13%), and septic shock (9%). Mortality was reported in three (13%) patients due to septic shock and ventricular fibrillation. CONCLUSIONS: Surgical mortality with the Bentall procedure is similar to published results by other specialized centers. Events related to the basic aortic pathology, surgical technique, aortic valve prosthesis and left ventricular dysfunction encourage longterm studies with follow-up.

Aneurisma del seno de Valsalva roto: informe de casos y revisión de la literatura / Sinus of Valsalva aneurysm with rupture: case report and literature review

Introducción: El aneurisma del seno de Valsalva es una dilatación que se origina por adelgazamiento de la unión de la capa media aórtica y el anillo fibroso; de origen adquirido y más frecuentemente congénito. El seno coronario derecho es el más afectado y la rotura es la complicación más común. Casos clínicos: Presentamos dos casos de aneurisma del seno de Valsalva roto a cavidades derechas con insuficiencia cardiaca severa, que constituyeron 0.4 % del total de cirugías cardiacas realizadas en un año en el Hospital de Especialidades, Centro Médico Nacional de Occidente, Guajalajara, Jalisco. Caso 1: hombre de 27 años con disnea de pequeños esfuerzos, disnea paroxística nocturna, ortopnea, fibrilación auricular paroxística, soplo diastólico, hepatomegalia y edema de miembros inferiores. Caso 2: hombre de 33 años con disnea de pequeños esfuerzos, disnea paroxística nocturna, ortopnea, soplo diastólico, insuficiencia renal aguda e insuficiencia hepática congestiva. La resolución quirúrgica consistió en resección del aneurisma y cierre del defecto con parche de dacrón, preservando la válvula aórtica. Ambos casos sobrevivieron. Conclusiones: La presencia de insuficiencia cardiaca y cambios auscultatorios súbitos obligan a sospechar aneurisma del seno de Valsalva roto. La ecocardiografía es suficiente para diagnosticar aneurisma del seno de Valsalva, sus complicaciones, repercusión y orientación quirúrgica. Cuando es asintomático existe riesgo de expansión, rotura, insuficiencia cardiaca, endocarditis, embolia y muerte súbita, lo que justifica la resolución quirúrgica, la cual tiene baja mortalidad a corto y largo plazo.

BACKGROUND: The sinus of Valsalva aneurysm (SVA) is a small dilatation caused by a separation between the aortic media and annulus fibrosus. Its origin may be either acquired or congenital. The right coronary sinus is most frequently affected, with the most common complication being rupture. CLINICAL CASES: We report two cases of SVA complicated with rupture to the right cavities with severe cardiac failure, which represents 0.4% of the total cardiac surgeries performed during the past year in our hospital. Case #1: We present the case of a 27-year-old male with low-effort dyspnea, nocturnal paroxysmal dyspnea, orthopnea, paroxysmal atrial fibrillation, diastolic murmur, hepatomegaly and edema of the lower extremities. Case #2: We present the case of a 33-year-old male with low-effort dyspnea, nocturnal paroxysmal dyspnea, orthopnea, diastolic murmur, severe kidney insufficiency and congestive hepatic insufficiency. Surgical resolution in both cases consisted of aneurysm resection and closure of the defect with a Dacron patch, preserving the aortic valve. Both patients survived. CONCLUSIONS: Cardiac failure and sudden auscultation changes suggest the possibility of SVA rupture. Echocardiography is sufficient to diagnose SVA, its complications, repercussions, and surgical options. SVA, even if asymptomatic, has potential risks of expansion, rupture, cardiac failure, endocarditis, embolism and sudden death. This justifies surgical correction, with a low mortality rate in both the short- and long-term.

Sinus of Valsalva aneurysm with rupture. Case report and literature review.

BACKGROUND: The sinus of Valsalva aneurysm (SVA) is a small dilatation caused by a separation between the aortic media and annulus fibrosus. Its origin may be either acquired or congenital. The right coronary sinus is most frequently affected, with the most common complication being rupture. CLINICAL CASES: We report two cases of SVA complicated with rupture to the right cavities with severe cardiac failure, which represents 0.4% of the total cardiac surgeries performed during the past year in our hospital. Case #1: We present the case of a 27-year-old male with low-effort dyspnea, nocturnal paroxysmal dyspnea, orthopnea, paroxysmal atrial fibrillation, diastolic murmur, hepatomegaly and edema of the lower extremities. Case #2: We present the case of a 33-year-old male with low-effort dyspnea, nocturnal paroxysmal dyspnea, orthopnea, diastolic murmur, severe kidney insufficiency and congestive hepatic insufficiency. Surgical resolution in both cases consisted of aneurysm resection and closure of the defect with a Dacron patch, preserving the aortic valve. Both patients survived. CONCLUSIONS: Cardiac failure and sudden auscultation changes suggest the possibility of SVA rupture. Echocardiography is sufficient to diagnose SVA, its complications, repercussions, and surgical options. SVA, even if asymptomatic, has potential risks of expansion, rupture, cardiac failure, endocarditis, embolism and sudden death. This justifies surgical correction, with a low mortality rate in both the short- and long-term.