Common arterial trunk for the branches of the third portion of the axillary artery: description of a case and its clinical application / Tronco arterial común para las ramas de la tercera porción de la arteria axilar: descripción de un caso y su aplicación clínica

SUMMARY: Variations of the axillary artery may have clinical implications capable of generating unexpected situations during surgical procedures of arterial reconstruction or vascular catheterization. The objective of this work was to report the finding of an anatomical variant of the axillary artery, which may have clinical and surgical implications. A descriptive study was conducted, in which a unilateral vascular variation found during a routine dissection in a right upper limb of a male cadaver was reported. From the second portion of the axillary artery originated a common arterial trunk that gave rise to the subscapular, anterior humeral circumflex, posterior humeral circumflex and deep brachial arteries. The third portion of the axillary artery did not emit branches. The common arterial trunk originated from the second portion, 62.64 mm from the beginning of the axillary artery. Its total length was 23.72 mm and its thickness was 6.1 mm. The caliber of the branches originating from the common arterial trunk was: subscapular artery 5.1 mm, anterior humeral circumflex of 1.66 mm, posterior humeral circumflex 3.18 mm and deep brachial 3.73 mm. The vascular variant detected altered the anatomical relationship of the axillary artery with the brachial plexus, generating a modification in the position of the fascicles and their terminal branches. Anatomical variations of the axillary artery are not infrequent, knowing them may be necessary during surgical procedures or anatomical dissections.

RESUMEN: Las variaciones de la arteria axilar pueden tener implicancias clínicas capaces de generar situaciones inesperadas durante procedimientos quirúrgicos de reconstrucción arterial o cateterismo vascular. El objetivo de este trabajo fue reportar el hallazgo de una variante anatómica de la arteria axilar, la cual puede tener implicancias clínicas y quirúrgicas. Se realizó un estudio de tipo descriptivo, en el cual se reportó una variación vascular unilateral encontrada durante una disección de rutina en un miembro superior derecho de un cadáver de sexo masculino. De la segunda porción de la arteria axilar se originó un tronco arterial común que daba origen a las arterias subescapular, circunfleja humeral anterior, circunfleja humeral posterior y braquial profunda. La tercera porción de la arteria axilar no emitía ramas. El tronco arterial común se originaba de la segunda porción, a 62,64 mm del inicio de la arteria axilar. Su longitud total era de 23,72 mm y su grosor de 6,1 mm. El calibre de las ramas originadas del tronco arterial común fue: arteria subescapular 5,1 mm, circunfleja humeral anterior de 1,66 mm, circunfleja humeral posterior 3,18 mm y braquial profunda 3,73 mm. La variante vascular detectada alteraba las relaciones anatómicas de la arteria axilar con el plexo braquial, generando una modificación en la posición de los fascículos y sus ramos terminales. Las variaciones anatómicas de la arteria axilar son frecuentes, conocerlas puede ser necesario durante procedimientos quirúrgicos o disecciones anatómicas.

Del soplo inocente a la hipertensión arterial pulmonar / From an innocent heart murmur to pulmonary arterial hypertension

Resumen Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Abstract Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

Persistent Truncus Arteriosus with Aortic Dominance in Female Adult Patient

Persistent truncus arteriosus categories associated with different natural histories and various surgical approaches were reported. Although pulmonary overflow and severe heart failure are common, some patients who have hypoplastic pulmonary artery systems may show lesser symptoms of heart failure and remain in relatively stable condition. We experienced a 33-year-old woman with uncorrected type II persistent truncus arteriosus who presented with cyanosis rather than congestive heart failure, and are presenting her images.

Variety of prenatally diagnosed congenital heart disease in 22q11.2 deletion syndrome

OBJECTIVE: To analyze the spectrum of prenatally diagnosed congenital heart disease in a Korean population with 22q11.2 deletion syndrome, and to provide guidelines for screening 22q11.2 deletion prenatally. METHODS: This retrospective study evaluated 1,137 consecutive fetuses that had prenatal genetic testing for 22q11.2 deletion because of suspected congenital heart disease between September 2002 and December 2012, at Asan Medical Center, Seoul, Korea. RESULTS: Main cardiovascular diseases in the 53 fetuses with confirmed 22q11.2 deletions were tetralogy of Fallot (n = 24, 45%), interrupted aortic arch (n = 10, 19%), ventricular septal defect (n = 5, 9%), double outlet right ventricle (n = 4, 8%), and coarctation of the aorta (n = 4, 8%). Other cardiac defects were rarely associated with 22q11.2 deletion. One fetus had persistent truncus arteriosus, one had aortic stenosis, and one had hypoplastic right heart syndrome. Two fetuses had normal intracardiac anatomy with an isolated right aortic arch, and one had an isolated bilateral superior vena cava. CONCLUSION: A variety of congenital heart diseases were seen during the prenatal period. Conotruncal cardiac defects except transposition of great arteries were strongly associated with 22q11.2 deletion. When such anomalies are diagnosed by fetal echocardiography, genetic testing for 22q11.2 deletion should be offered. Even if less frequent deletion-related cardiac defects are detected, other related anomalies, such as thymic hypoplasia or aplasia, should be evaluated to rule out a 22q11.2 deletion.

Truncus arteriosus: surviving at 46 years without intervention / Tronco arterioso: sobreviviendo a los 46 años sin intervención

The median survival of patients with truncus arteriosus is five weeks of age with rare cases reaching adulthood. The longest reported survivor with balanced ventricles, as the index case has, is 38 years of age. The index case is a 46-year old male with Type 3 truncus arteriosus. His case has never been documented in the English Literature as far as the author is aware.

La mediana de la supervivencia de pacientes con tronco arterioso es cinco semanas de edad, siendo raros lo casos que alcanzan la edad adulta. El caso más viejo reportado con ventrículos balanceados, como en el caso índice, es un sobreviviente de 38 años de edad. El caso índice es un hombre de 46 años con tronco arterioso de tipo 3. Su caso nunca se ha documentado en la literatura en inglés, hasta donde el autor tiene noticias.

Aortopathy in Congenital Heart Disease in Adults: Aortic Dilatation with Decreased Aortic Elasticity that Impacts Negatively on Left Ventricular Function

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".

Surgical repair of truncus arteriosus in children: early results and long-term outcomes / 中华外科杂志

<p><b>OBJECTIVE</b>To recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA).</p><p><b>METHODS</b>The clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively. There were 36 male and 18 female patients, with a mean age of (9 ± 10) months (range, 1 to 38 months; median, 5 months). Preoperative mechanical ventilation was required in 5 patients. The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery. The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases), or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA).</p><p><b>RESULTS</b>There were 3 patients (5.6%) died of pulmonary hypertensive crisis in hospital. The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation, while the others were 3.6 days. Forty-seven (92.2%) patients were followed-up for mean (6.8 ± 2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation. One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively. Among those patients who underwent direct anastomoses, 8 (32.0%) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0%) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively. Among those inserting conduits, 7 patients (31.8%) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality.</p><p><b>CONCLUSIONS</b>It is difficult to treat the PTA patients with IAA, intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT, but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.</p>

Tronco común: variantes anatómicas, tratamiento quirúrgico y evolución / Truncus arteriosus, anatomy, surgical treatment, follow up

OBJECTIVE: To evaluate through a retrospective cohort the anatomy and results of patients that were operated of truncus arteriosus. MATERIAL AND METHODS: Historic cohort. From January 2000 to December 2005 twenty eight patients with troncus arteriosus were operated. RESULTS: There were fifteen male (53.6%) and 13 female patients (46.4%). The median age, at the time of the surgery, was 10.5 months. The median weight, at the time of the surgery was 6 kg. All the patients were dominant aortic. A woven-dacron tube was used in 25 cases, Hanckock to 2 and Barbero Marcial procedure in one. The media diameter for the tube for the 27 patients (96.4%) was of 14 +/- 2 mm. Two patients required aortic valvular replacement at the same time of the surgery and two more during follow up, after the first surgery. Fifty per cent of the patients had pulmonary hypertension crisis after de surgery. There were three deaths: one patient had cardiogenic shock; another had pulmonary hypertension and one more had obstruction of the traqueostomy cannula. During study monitoring, 3 patients (10.7%) needed interventionist procedures. One case needed dilatation of the tube and two cases needed dilatation of the pulmonary branches to implant a stent device. There has been one tube change 4.2 years after the correction. Actuarial survival after 30 days was of 96.42%, and 88.9% after one and five years. CONCLUSIONS: The surgical correction of the truncus arteriosus has allowed changing the natural history of this disease. Mortality is low however our follow up is not very long yet.

Atresia tricuspídea asociada a tronco común: reporte de un caso / Tricuspid atresia associated with common trunk: a case report

The tricuspid atresia associated with persistent truncus arteriosus is a rare anomaly. A case is presented of one male patient of three months age in which was detected a cardiac murmur. The diagnosis was made by echocardiography and confirmed by cardiac catheterization. The first step of the palliation was done with disconnection of the trunk of the pulmonary artery combined with a systemic- to- pulmonary shunt of 4 mm. The patient died 24 hours later for persistent metabolic acidosis.

Tronco arterioso común en adultos / Common truncus arteriosus in adults

The purpose of this study is to describe the factors that allow for the survival of patients with persistent truncus arteriosus until adult life and cared for at the National Institute of Cardiology [quot ]Ignacio Chávez[quot ]. From May 2003 to June 2007, six adult patients with clinical suspicion of truncus arteriosus were studied. All patients were subjected to clinical history, electrocardiogram of twelve leads, echocardiography, and cardiac catheterism. In these patients, the clinical symptoms began early of life. The ecocardiographic findings demonstrated the presence of truncus arteriosus type I in all cases and the most frequent associated anomalies were: hypoplasia of pulmonary branches, isolated coronary ostium, persistent ductus arteriosus and right aortic arch. Almost all patients had pulmonary hypertension. Based on this work, we conclude that until now we do not known the actual survival time of adult patients with complex congenital heart disease, such as truncus arteriosus. There are factors, such as pulmonary hypertension and hypoplasia of the pulmonary branches that are deleterious for life in some cardiovascular diseases, but in others allow for the survival of patients, as occurred in these patients with truncus arteriosus.

Anestesia en el tronco arterioso común / Anesthesia in the common truncus arteriosus

El tronco arterioso es una anomalía congénita del corazón poco frecuente, que se asocia con una alta mortalidad. El 50 por ciento de los pacientes fallece antes de los 3 meses de edad y el 80 por ciento antes del año. La causa más frecuente de muerte es el fallo cardíaco agudo. Presentamos una experiencia en 2 lactantes operados en el Cardiocentro del Hospital Pediátrico Universitario William Soler, durante el año 2001: una niña de 7 meses de edad, desnutrida, con un tronco común tipo II, hipertensión pulmonar y síndrome de DiGeorge, el segundo paciente era un lactante de 3 meses de edad, con un tronco común tipo I. Se utilizó fentanyl en la inducción y el mantenimiento de la anestesia. La temperatura recta se bajó a 25 °C y los tiempos de circulación extracorpórea fueron de 100 y 121 min y el de pinzamiento aórtico de 30 y 31 min. Se utilizó apoyo farmacológico con dobutrex en dosis bajas. Se emplearon fentanyl, vasodilatadores en infusión continua e hiperventilación, como medidas para evitar la crisis de hipertensión pulmonar. Los enfermos evolucionaron satisfactoriamente durante todo el transoperatorio.

Bilateral percutaneous stent implantation in the pulmonary vasculature: a modality now available in Lebanon

We report a child with Truncus Arteriosus who developed bilateral proximal branch pulmonary stenosis, following total surgical repair of her condition with construction of the right ventricular outflow tract utilizing a Contegra conduit [Medtronic. Inc., Minneapolis, Minn.]. The obstruction was relieved completely utilizing bilateral percutaneous stent implantation. To the best of our knowledge, this is the first reported case of bilateral stent implantation to relieve branch pulmonary artery stenosis in Lebanon. This technique could he applied to similar cases of obstruction in the pulmonary tree

Correction of truncus arteriosus using a fresh autologous pericardial trileaflet valve conduit.

A fresh autologous pericardial trileaflet valve conduit was used in a 5-month-old infant for correction of truncus arteriosus. The patient recovered from the operation with satisfactory hemodynamics and post-operative echocardiogram at 3 months showed only mild to moderate pulmonary regurgitation. This technique is a useful alternative for correction in an infant with congenital heart disease who needs a tissue valve conduit. It may be more suitable than an aortic homograft by reason of unavailability of small homograft and limitation of organ donation.

Persistent Truncus Arteriosus with Survival to the Age of 29 Years

The Truncus arteriosus is a congenital malformation in which only one great artery arises from the base of the heart and gives origin to the systemic, pulmonary and coronary arteries proximal to the aortic arch. Pulmonary blood flow is governed by the size of the pulmonary arteries and the pulmonary vascular resistance. In infancy, pulmonary blood flow is usually excessive because pulmonary vascular resistance is not greatly increased. Thus, despite an obligatory admixture of systemic and pulmonary venous blood in the common trunk, only minimal cyanosis is present. Rarely pulmonary blood blood flow is restricted by hypoplastic or stenotic pulmonary arteries arising from the truncus. The prognosis in persistent truncus arteriosus is very poor. the median age of survival of the 94 patients with the disease reported up to 1962 was only five weeks. The longest survival reported is the case of the man described by Carr et al who lived to the age of 36 years and 2 months. We report the case of a man with persistent truncus arteriosus who lives to the age of 29 years.