A case report of Ebstein's anomaly treated with Carpentier's procedure

A 42-year-old male patient was admitted with congestive heart failure. Echocardiogram and cardiac angiogram revealed Ebstein's anomaly and severe tricuspid valve incompetence. He underwent Carpentier's procedure for the treatment of Ebstein's anomaly. Carpentier's procedure consists of the following two new points; (1) the right atrium and atrialized ventricle is plicated longitudinally; (2) the anterior leaflet and the posterior leaflet of tricuspid valve are transposed with a clockwise rotation to the level of the normal tricuspid anulus. Compared with Hardy's procedure (transverse plication), longitudinal plication preserves the cavity and the function of the right ventricle and excludes atrialized chamber. In our case, there is a trivial residual regurgitation of tricuspid valve postoperatively, but the patient's clinical status has improved remarkably. We conclude that Carpentier's procedure is an effective operation for Ebstein's anomaly.     

A case report of Ebstein's anomaly treated with Hetzer's procedure

A 27-year-old male who had been diagnosed with Ebstein's anomaly was admitted with uncontrollable congestive heart failure. The echocardiogram revealed severe tricuspid valve incompetence and the electrocardiogram showed atrial fibrillation. He underwent Hetzer's repair procedure for tricuspid valve incompetence and Minzioni's right atrial isolation technique to restore sinus rhythm. His congestive heart failure quickly disappeared and sinus rhythm was restored after operation. He was discharged 3 weeks postoperatively and remains well 22 months after his operation. Hetzer's technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. We conclude that Hetzer's procedure is an effective operation for Ebstein's anomaly.     

Ebstein's anomaly in an adult

A 56-year-old female who had been diagnosed with Ebstein's anomaly was admitted with cyanosis and congestive heart failure. The echocardiogram showed severe tricuspid valve incompetence, displacement of the tricuspid valve and dilatation of the atrialized portion of the right ventricle. Atrial fibrillation was detected in the electrocardiogram. She underwent tricuspid valve replacement and right atrial maze procedure. She is released from congestive heart failure and remains in sinus rhythm 48 months after the operation.     

Pheochromocytoma in an elderly patient: report of a case

This is a case report of a pheochromocytoma which developed in a 67-year-old man. The patient presented himself with a productive cough and orthopnea, both of which were subsequently proved to be due to hypertensive heart failure. The diagnosis of a pheochromocytoma originating from the left adrenal gland was established endocrinologically and roentgenologically. Transperitoneal adrenalectomy was undertaken, and a tumor weighing 300 g was obtained. Histopathologically, the tumor was composed of two elements: cells with profuse cytoplasma having chromaffin-positive granules, and other cells consisting of spindle cells with mitosis. Surgical exploration could not identify another tumor or metastasis, and his blood pressure returned to normal, with normal catecholamine levels, after surgery. This is the first reported case of an elderly person with a pheochromocytoma complicated by congestive heart failure and renal insufficiency preoperatively; however, it was controlled well, and he underwent surgery successfully. This case constitutes the 64th report on a pheochromocytoma in persons over 60 years of age in the Japanese literature.     

Intralobar sequestration in elderly patient; report of a case

A 71-year-old woman who had been treated as bronchitis was diagnosed as intralobar sequestration by a computed tomography scan. She had 2 episodes of hemosputum in recent 30 years but the amounts were small and they spontaneously stopped in a day. She had an abnormal vessel from thoracic descending aorta of which diameter was 1 cm draining to the basal segment of the left lung. She was operated on a left lower lobectomy with ligation of the vessel via a thoracotomy. Postoperative course was uneventful, and she discharged home on the 9th postoperative day.     

Left ventricular free wall rupture in an elderly patient; report of a case

We successfully repaired left ventricular free wall rupture in a 91-year-old woman. She was admitted to our hospital because of consciousness loss. Blood pressure was only 60 mmHg on arrival. Contrast-enhanced computed tomography (CT) demonstrated massive pericardial effusion and transmural contrast defect of the left ventricular lateral wall in the delayed phase. Upon surgery there was massive clot around the heart. A localized infracted area on the left ventricular lateral wall was exposed using off-pump coronary artery bypass (OPCAB) technique. There was mild bleeding only during diastolic phase. Three mattress sutures of 3-0 prolene with Teflon felt strips were placed to control the bleeding under beating heart. A sheet of collagen and fibrin glue was also applied. Postoperative course was uneventful. This type of left ventricular rupture could be less invasively repaired by off-pump technique. As far as we investigated, this is the oldest patient of ventricular free wall rupture successfully repaired.     

Repair of septal and posterior tricuspid leaflets in Ebstein's anomaly

BACKGROUND AND AIM: In Ebstein's anomaly, the septal and posterior tricuspid leaflets are plastered to the endocardium. We postulated that tricuspid valve function could be corrected by restoring mobility of these leaflets. (Feasibility of such repair was explored by anatomical and clinical studies.) METHODS: Ten heart specimens with Ebstein's anomaly were examined to investigate the size of the tricuspid leaflets. We operated on four patients with Ebstein's anomaly: the plastered septal and posterior leaflets were mobilized from the endocardium, the atrialized right ventricle was longitudinally plicated, and the basal attachment of the mobilized leaflets was sutured (reattached) to the valve annulus. RESULTS: In heart specimens, approximately 40% of the total surface of the tricuspid leaflets was comprised of the septal and posterior leaflets. Clinically, all patients operated on returned to normal functional status after surgery. The mean cardiothoracic ratio on chest X-rays decreased from 0.70 to 0.55 (after surgery). Echocardiographic tricuspid regurgitation, graded from 0 to 4, decreased from 3.5 to 1.0, and tricuspid annular diameter ratio to the normal value reduced from 1.88 to 0.66. Angiographic right ventricular ejection fraction increased from 0.36 to 0.50, and end-diastolic volume ratio to the normal value decreased from 3.65 to 1.19. CONCLUSIONS: Repair of the septal and posterior tricuspid leaflets was found to be feasible and effective as tricuspid valvuloplasty for Ebstein's anomaly.     

Anesthesia for cesarean section in a patient with Ebstein's anomaly]

A 30-year-old woman with Ebstein's anomaly was scheduled for Cesarean section at 38 week gestation because of latent fetal distress. After arterial and central venous catheters were inserted, general anesthesia was started. Anesthesia was induced smoothly and rapidly through intravenous route. Following induction, her hemodynamics was stable until post-operative period. The baby weighed 1564g and had an Apgar score of 8 at 1 minute. A patient with Ebstein's anomaly during pregnancy and anesthesia has potential for a variety of hemodynamic disturbances. This case illustrates the importance of careful attention to the preoperative findings and the perioperative hemodynamic parameters.     

An unusual first presentation of Ebstein's anomaly in a 72-year-old patient

We report the unusual case of a 72-year-old woman with a primary presentation of right heart failure in the setting of recently diagnosed Ebstein's anomaly with concomitant atrial fibrillation (AF). The patient had New York Heart Association (NYHA) class III dyspnea for 12 months, with refractory right heart failure prior to undergoing surgical management in the form of a tricuspid annuloplasty ring and plication of the atrialized ventricle. The patient had an uneventful postoperative recovery and enjoyed an improvement in her exercise tolerance (NYHA class I) with minimal echocardiographic evidence of tricuspid regurgitation (TR) at the latest follow-up.     

Asymptomatic balanced-type double aortic arch in an elderly patient: a case report

Double aortic arch is a congenital abnormality and sporadic cases have been reported in adult patients, who are usually diagnosed after complaining of asthma-like symptoms or swallowing difficulties because of the compression of the trachea or esophagus by the abnormal aortic arches. We present the case of a 67-year-old male patient with double aortic arch, found coincidentally during coronary angiographic examination.     

Multiple complications following radial keratotomy in an elderly patient: a case report

A 68-year-old female underwent a 16-incision radial keratotomy in 1980. Her post-op course was complicated by marked overcorrection (+11.00), irregular astigmatism, and endothelial cell loss of almost 50%. Radial keratotomy in the elderly should be approached with caution because of the apparent increased effect of the surgery that occurs with increasing age.     

Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up

BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates. We now update our experience with emphasis on the evolution of our surgical technique and the medium-term follow-up of these patients. METHODS: Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebstein's anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs. RESULTS: One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old. CONCLUSIONS: Surgical repair of the severely symptomatic neonate with Ebstein's anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.     

Pilocytic astrocytoma of the cerebrum presenting in an elderly patient: a case report

We report a case of pilocytic astrocytoma of the cerebrum presenting in an elderly patient. A 76-year-old man was admitted to our department due to the development of dysarthria. MRI showed a cystic mass with an enhanced small mural nodule in the left frontal lobe. At surgery, the cyst contents were aspirated, and the mural nodule was excised. Histological examination showed a pattern that is usually seen in pilocytic astrocytoma of the cerebellum, including loose and compact areas composed of pilocytic and stellate cells, a few eosinophilic granular bodies, but not Rosenthal fibers. Pilocytic astrocytoma is a common type of pediatric brain tumor that can arise within either the cerebellum or the hypothalamic/chiasmatic region, but rarely seen in the cerebral hemisphere at an advanced age. To our knowledge, only 45 cases of pilocytic astrocytoma of the cerebrum developing in an adult are reported. In those cases, the symptoms of the disease developed during the third decade of life. The onset at a most advanced age as in the present case is thought to be extremely rare.     

Split spinal cord malformation in an elderly patient: case report

Split spinal cord malformations (SCM) typically present in childhood and rarely in adulthood. Very little is known about the SCMs in the elderly, and the diagnosis can be easily missed. A 73-year-old woman with a childhood history of scoliosis and late ambulation milestones presented with a 2-year history of worsening low back pain and progressive difficulty walking. She had a mild gait disturbance with 4/5 weakness in left ankle dorsiflexion. Magnetic resonance imaging revealed a bifid spinal cord contained in a single thecal sac and a tethered cord with low-lying conus at L3. The patient was taken to the operating room and a soft-tissue median septum, as well as all other adhesions, was removed. The filum terminale was identified, coagulated, and divided. Six weeks later, the patient reported decreased back pain, improvement in ambulation, and markedly decreased used of narcotics for her back and leg pain. Her left ankle dorsiflexion strength improved to 4+/5. This patient had two hemicords encased in a single dural tube separated by a nonrigid, fibrous median septum and an associated tethered cord. Adult presentation of SCM is extremely rare. This case highlights the need to consider split cord malformation and tethered cord in the differential diagnosis not only for adults but also the elderly presenting with back pain, scoliosis, and difficulty walking.     

Reconstruction of the right heart in an elderly woman with Ebstein's anomaly and severe heart failure

We report the case of a 53-year-old woman with Ebstein's anomaly and intractable heart failure who had undergone only tricuspid valve replacement 30 years earlier. She was treated conservatively for 1 month; however, she was placed in the New York Heart Association functional class IV. Therefore, we operated on her with the objective of improving her quality of life and cardiac function. One-and-a-half repair and a second tricuspid valve replacement with right ventriculoplasty were quite effective in ameliorating her critical condition.