Diaphragmatic Defect with Peritoneopericardial Communication

Abstract

An 81-year-old man had a congenital defect of the central tendon of the diaphragm, including a peritoneopericardial communication with herniation of the omentum to the pericardial sac in front of the heart. In addition, he had a critically stenosed congenital bicuspid aortic valve and severe coronary artery disease. The patient underwent reduction of the herniated omentum into the abdominal cavity, coronary artery bypass grafting, aortic valve replacement, and closure of the peritoneopericardial communication with a synthetic patch. Three years later, the patient was doing well, with a normally functioning pericardial valve in the aortic position and no sign of omentum around the heart. (Tex Heart Inst J 2003;30:328–31)

A congenital diaphragmatic hernia occurs in 1 of 2,100 to 1 of 5,000 births, ¹ and a congenital bicuspid aortic valve (BAV) occurs in 1% to 2% of the population. ² Among all congenital diaphragmatic hernias, a defect of the central tendon of the diaphragm with a peritoneopericardial communication (PPC), whether congenital or traumatic, is rare. ^1,3 The incidence of concomitant congenital anomalies, particularly of the nervous system and the heart, ranges from 20% to 30% in newborns with congenital diaphragmatic hernia.

Case Report

In early February 2000, an 81-year-old man began experiencing transient episodes of syncope and palpitations. In July of 1932, at the age of 13, he had been shot with a 22-mm-caliber gun. The entry of the bullet was 3 cm below the left nipple; there was no point of exit. While the patient was in the hospital, the bullet seemed to present no problems and was therefore left alone. Subsequently, from 1944 to 1954, the man was physically fit for military service and had served as a sergeant in the United States Army—a fact which indicates that he had remained asymptomatic.

Since early February 2000, when the patient had begun experiencing syncope and palpitations, he had been in a state of controlled atrial fibrillation, taking di-goxin, 0.125 mg daily, and warfarin sodium, 5 mg daily. When he presented at our institution in September 2000, his heart rate was 80 beats/min and irregular. His blood pressure was 110/60 mmHg, and on auscultation there was a harsh grade 3/6 systolic murmur in the aortic area radiating to both carotid arteries and into the apex of the heart. Chest radiography revealed marked enlargement of the left ventricle and the presence of a 22-caliber bullet, 2.5 cm to the left and posterior to the esophageal hiatus of the diaphragm.

Transthoracic echocardiography (15 February and 12 June 2000) demonstrated an atherosclerotic narrowing of the congenital BAV, with fusion of the right and left leaflets, a valve area of 0.7 cm², a left ventricular ejection fraction of 0.35, and mild pulmonary hypertension (pulmonary artery systolic pressure, 45 mmHg). The parasternal short-axis view of the aorta revealed a 2-cm space between the anterior wall of the right ventricle and the pericardium, findings that were compatible with a herniated omentum (Fig. 1).

Fig. 1 Transthoracic echocardiogram of the aorta (12 June 2000), parasternal short-axis view, shows a 2-cm space between the anterior wall of the right ventricle and the pericardium, compatible with a herniated omentum.

Cardiac catheterization (19 September 2000) showed a 90% narrowing of the proximal left anterior descending artery and its diagonal branch, and confirmed the presence of severe aortic valve stenosis: peak systolic gradient, 70 mmHg; mean gradient, 52 mmHg; and valve area, 0.7 cm². The patient had a history of deep venous thrombosis and had undergone insertion of an inferior vena cava filter. Consequently, right-heart catheterization could not be performed because of the presence of the inferior vena cava filter and the thrombosed vein below the filter.

Surgical Technique

Surgery was performed on 4 October 2000, with the aid of transesophageal echocardiography, which confirmed high-grade stenosis of the congenital BAV and showed no evidence of atheromatous changes in the ascending aorta. The intrapericardial space was not seen.

After performing a median sternotomy and opening the pericardium longitudinally, we saw the omentum protruding through a round 10- × 10-cm defect of the central tendon of the diaphragm, which constituted a PPC. The omentum covered the entire heart, the ascending aorta, and the main pulmonary artery (Fig. 2). The hernia had no sac, and there were no adhesions to the pericardium, heart, ascending aorta, or main pulmonary artery. After the omentum was returned to the abdominal cavity, we could see the liver, the stomach, the transverse colon, and the spleen, through the PPC of the diaphragm (Fig. 3). The defect of the central tendon of the diaphragm was temporarily closed with a laparotomy sponge.

Fig. 2 Photograph after a median sternotomy and longitudinal opening of the pericardium shows the omentum, herniated through a defect in the central tendon of the diaphragm and through a peritoneopericardial communication, completely covering the heart, the ascending aorta, and the main pulmonary artery.

Fig. 3 After reduction of the omentum that protruded from the peritoneal cavity into the pericardium, the posterior margin of the peritoneopericardial communication of the diaphragm is visible, as well as the liver, stomach, transverse colon, and spleen.

The operation was performed using a standard technique. The coronary artery bypass, with use of a sequential vein graft to the left anterior descending artery and its diagonal branch, was followed by excision of the stenotic congenital BAV, aortic valve replacement with a No. 23A pericardial valve, and closure of the defect of the central tendon of the diaphragm with a 10- × 10-cm synthetic patch and a continuous 3-0 polypropylene suture (Fig. 4).

Fig. 4 The defect in the central tendon of the diaphragm and a peritoneopericardial communication were closed with a 10- × 10-cm synthetic patch.

The patient's postoperative course was smooth. He remained in controlled atrial fibrillation; therefore, warfarin was resumed at the preoperative dosage. Transthoracic echocardiography, performed on 14 May 2003, showed a normally functioning pericardial valve in the aortic position and no omentum around the heart. The patient, when last seen in August 2003, was doing well.

Discussion

A congenital diaphragmatic hernia can include any of the following: an eventration of the diaphragm, a posterolateral hernia through the lumbocostal triangle of Bochdalek, a hernia through the right retrosternal triangle of Morgagni or the left sternocostal triangle of Larrey, or a hernia through the defect in the tendinous part of the diaphragm (the central tendon of the diaphragm, central tendineum diaphragmae) with a PPC. ¹

The central tendon of the diaphragm, which originates from many different mesenchymal sources (including the septum transversum), is a thin but strong aponeurosis of closely interwoven fibers. It is located in the central anterior portion of the diaphragm and resembles a cloverleaf in shape, with 1 anterior and 2 lateral leaves; the largest expansion is in the transverse plane. The upper surface of the central tendon and the lower surface of the pericardium are partially blended. The weight of the heart makes a downward indentation in the central tendon, which is visible on chest radiographs.

Occasionally in women, during the 4th month of pregnancy, the central tendon of the diaphragm ruptures, forming a PPC that subsequently gives rise to a hernia. ⁴ The sac is seldom seen in that type of hernia.

A congenital defect of the central tendon of the diaphragm with a PPC is very rare. The male-to-female ratio is unknown. The 1st signs or symptoms of this condition can occur at any age. It has been seen in newborns and in adults. A defect of the central tendon of the diaphragm with a PPC is associated with ectopia cordis and with an atrial septal defect (only 3 patients reported). ^4,5 Herniation in the pericardial cavity may be asymptomatic or may manifest as cardiac dysfunction due to compression and respiratory distress. ^1,4,5

Since our patient had a defect of the central tendon of the diaphragm with a PPC and a BAV, it is very likely that both were of congenital origin. In patients with a BAV, both the valve and the media of the ascending aorta contain less elastic tissue—specifically, the elastic lamellae are thinner and more dispersed. ⁶ It is conceivable that the embryonal centrum tendineum diaphragmae also contains less elastic tissue and that this contributes to the development of the defect.

In 980 patients with 1,000 injuries resulting from blunt or penetrating diaphragmatic trauma, hernias occurred as follows: in the left hemidiaphragm, 685 (68.5%); in the right hemidiaphragm, 242 (24.4%); bilateral, 15 (1.5%); in the central tendon of the diaphragm with a PPC, 9 (0.9%); and unclassified, 49 (4.9%). ³

Traumatic diaphragmatic hernias are characterized by 3 phases: acute, latent, and late (obstructive). 1,3,7,8 Clinical features of the acute phase are abdominal pain, respiratory dysfunction, and cardiac dysfunction. The latent phase manifests as pain in the left upper quadrant, chest, or left shoulder; dyspnea; and decreased breath sounds. In the late phase, prominent are nausea and vomiting, abdominal pain, and a mediastinal shift with respiratory and cardiac dysfunction.

Our patient had sustained a 22-mm-caliber bullet injury to the left chest with lodgment at the left of the esophageal hiatus of the diaphragm at an early age. The fact that there was no cardiac or respiratory dysfunction thereafter would indicate that the bullet did not cause the defect of the central tendon of the diaphragm with the PPC and herniation of the omentum into the pericardial sac.

One can assume that, in this patient, a congenital defect of the central tendon of the diaphragm with a PPC was associated with a congenital BAV. A traumatic origin of the defect from the 22-mm-caliber bullet was unlikely, particularly in the absence of any subsequent signs or symptoms. To our knowledge, there is no previous report of an association between a congenital defect of the central tendon of the diaphragm with a peritoneopericardial communication and a congenital bicuspid aortic valve.