Cantrell Syndrome


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Circ J. 2003 Nov;67(11):972-4

Multiple left ventricular diverticula detected by second harmonic imaging.

Takahashi M, Nishikimi T, Tamano K, Hara S, Kobayashi T, Honda T, Uetake S, Mitobe R, Matsuoka H.

Department of Hypertension and Cardiorenal Medicine, Dokkyo University School of Medicine, Mibu.

A 51-year-old Japanese man was admitted to hospital for evaluation of anterior chest pain at rest. He had a past history of cerebellar infarction when he was 47 years old. A conventional 2-dimensional echocardiogram revealed normal left ventricular function, and no abnormal findings. However, second harmonic imaging demonstrated 2 noncontractile diverticula at the submitral annular portion of the posterior wall of the left ventricle. Second harmonic imaging revealed another contractile diverticulum at the anterior wall of left ventricle in the short-axis view. These findings were confirmed by left ventriculography. After confirming the diagnosis of multiple left ventricular diverticula, a rare type of congenital anomaly, anticoagulant therapy was started and he is well to date. Left ventricular diverticula are clinically very important because they are often associated with serious complications such as systemic thromboembolism. Enhancement of the left ventricular endocardial borders by second harmonic imaging is useful for the diagnosis of left ventricular diverticula.


Echocardiography. 2003 Feb;20(2):191-5

Asymptomatic isolated congenital left ventricular muscular diverticulum in an adult: a case report.

Huang G, Pavan D, Antonini-Canterin F, Piazza R, Burelli C, Nicolosi GL.

Unita Operativa di Cardiologia, A.R.C., Azienda Ospedaliera S. Maria degli Angeli, Via Montereale 24, 33170 Pordenone, Italy. cardiologia@aopn.fvg.it

Congenital ventricular diverticulum is a very rare malformation in adults. We describe a 21-year-old male with a congenital muscular left ventricular diverticulum in the inferior wall. The lesion was suspected on two-dimensional transthoracic echocardiography; transesophageal echocardiography allowed clear detection of the diverticulum as well of mild mitral valve prolapse. The diagnosis was confirmed by cardiac catheterization. There were no other thoracoabdominal or cardiac anomalies, the patient was asymptomatic, and surgery was not deemed necessary.


Acta Cardiol. 2003 Jun;58(3):207-8

A congenital left ventricular diverticulum combined with a complex malformation syndrome.

Massin MM, Verloes A.

Division of Paediatric Cardiology, University of Liege at CHR Citadelle, Belgium. martial.massin@chrcitadelle.be

Congenital left ventricular diverticulum is rare and usually isolated or associated with midline thoracoabdominal defects and other congenital cardiac malformations. We describe a case with a previously undescribed malformation syndrome including bilateral obstruction of the ureteropelvic junction, agenesis of corpus callosus, scoliosis, hip dysplasia, severe developmental delay, failure to thrive and congenital left ventricular diverticulum but with normal GTG-banded karyotype. The cardiac lesion was suspected on echocardiography and was confirmed by cineangiography, which outlined a huge apical diverticulum opening through a wide ostium into the left ventricular cavity. To our knowledge this case report is the first description of an association with such a malformation syndrome.


Ann Cardiol Angeiol (Paris). 1999 Jan;48(1):13-5

Congenital left ventricular aneurysms and diverticula. Two case reports

Bire F, Rouxel MF, Chevalier JM, Quiniou G, Guerin P, Roques X, Choussat A.

Service de Cardiologie, Hopital d'Instruction des Armees R. Picque, 33998 Bordeaux Armees.

The authors report the cases of two young adults (25 and 27 years) presenting with congenital left ventricular aneurysm or diverticulum with healthy coronary arteries. This saccular evagination of the ventricular wall is rare. The authors describe a classification distinguishing muscular (contractile) diverticula, composed of the three cardiac tunics, fibrous diverticula and finally aneurysms with a dyskinetic wall. Although the limits of this classification are sometimes poorly defined, it presents a prognostic value, because it appears logical to propose nonsurgical management in the context of muscular diverticula and it seems legitimate to operate on fibrous diverticula and aneurysms due to the risks of rupture, extension, thrombosis or arrhythmia.


J Formos Med Assoc. 2002 Oct;101(10):729-31

Diverticulum of the right ventricle associated with pulmonary stenosis, rhabdomyoma and Wolff-Parkinson-White syndrome.

Yao CT, Wang JN, Yang YR, Tsai YC, Wu JM.

Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.

Congenital cardiac diverticulum is a rare anomaly that may present as an isolated lesion or in association with other malformations. Diverticulum of the left ventricle is more common than that of the right ventricle. We report a case of cardiac diverticulum over the right ventricular outflow tract and associated pulmonary stenosis, right atrial rhabdomyoma, and Wolff-Parkinson-White syndrome in a 9-month-old boy. The delta wave disappeared after removal of the atrial rhabdomyoma.


Angiology. 2001 May;52(5):357-9

Contrast harmonic power Doppler imaging of congenital ventricular diverticulum--a case report.

Ueda T, Mizushige K, Yukiiri K, Aoyama T, Kondo I, Kohno M, Matsuo H.

Second Department of Internal Medicine, Kagawa Medical University, Japan.

A case of an 81-year-old woman with a left ventricular diverticulum who underwent myocardial contrast echocardiography is reported. After administration of the contrast agent, a pulsed Doppler flow measurement clearly revealed the biphasic waveform of the ejection flow in the pre-systolic and systolic phase at the ostium of the diverticular cavity. A harmonic power Doppler image showed that part of the diverticulum wall had similar acoustic properties to the ventricular septal wall. Intracardiac blood flow and myocardial perfusion could be clearly evaluated and a ventricular diverticulum was correctly diagnosed using contrast echocardiography.


Angiology. 2001 May;52(5):357-9

Contrast harmonic power Doppler imaging of congenital ventricular diverticulum--a case report.

Ueda T, Mizushige K, Yukiiri K, Aoyama T, Kondo I, Kohno M, Matsuo H.

Second Department of Internal Medicine, Kagawa Medical University, Japan.

A case of an 81-year-old woman with a left ventricular diverticulum who underwent myocardial contrast echocardiography is reported. After administration of the contrast agent, a pulsed Doppler flow measurement clearly revealed the biphasic waveform of the ejection flow in the pre-systolic and systolic phase at the ostium of the diverticular cavity. A harmonic power Doppler image showed that part of the diverticulum wall had similar acoustic properties to the ventricular septal wall. Intracardiac blood flow and myocardial perfusion could be clearly evaluated and a ventricular diverticulum was correctly diagnosed using contrast echocardiography.


Scand Cardiovasc J. 2001 Feb;35(1):55-7

Left ventricular diverticulum associated with Cantrell's syndrome and tetralogy of Fallot in an adult.

Alayunt A, Yagdi T, Alat I, Posacioglu H, Buket S.

Ege University Medical Faculty, Department of Cardiovascular Surgery, Bornova, Izmir, Turkey. alayunt@med.ege.edu.tr

In this report we describe the surgical treatment of a 27-year-old patient with complete Cantrell's syndrome, i.e. multiple ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with tetralogy of Fallot. Resection of the diverticulum was combined with correction of the tetralogy of Fallot and thoracoabdominal defects. The postoperative period was uncomplicated. We have have found only one previous report describing resection of the diverticulum combined with correction of Fallot's tetralogy and thoracoabdominal defects in an adult. One-stage repair of these complex anomalies is technically feasible and should be the treatment of choice.


Ann Thorac Surg. 1998 Apr;65(4):1178-85

Cantrell's syndrome: a challenge to the surgeon.

Vazquez-Jimenez JF, Muehler EG, Daebritz S, Keutel J, Nishigaki K, Huegel W, Messmer BJ.

Thoracic and Cardiovascular Surgery and Pediatric Cardiology, Rheinisch-Westfalische-Technische Hochschule Aachen, Germany.

We present a case of partial Cantrell's syndrome with ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with omphalocele. At the age of 20 months, the patient underwent a successful cardiac surgical procedure. To detect risk factors and to define therapeutic strategies, we analyzed the spectrum and the frequency of malformations described in 153 patients with Cantrell's syndrome. Despite modern surgical standards, Cantrell's syndrome represents a challenge to the surgeon because of the wide spectrum of anomalies, the severity of the abdominal and cardiac malformations, and the high mortality.


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