Friday, July 31, 2009

Welcome back to our voyage called History of Pediatric Cardiology.

Getting back to the Congenital heart lesions, we have seen some of the works by Steno, Morgagni and others, which led to the development of interest in the field. In the 18th century, a Frenchman by name LeCat brought out a study on Atrial Septal Defects in AD 1747, with a quality that was unparalleled till then and set a trend for future. Also seen was a lively debate (which used to run toxic if opposite parties met!) on the origin of cyanosis – obstruction Vs admixture. Obstruction to the flow theory, vehemently supported by Thomas Peacock cited instances of cyanosis in the absence of septal defects. The other group which supported the admixture lesions was physicians like Gintrac, who quoted patients of pulmonary stenosis who had no cyanosis at all, thereby arguing in favor of admixture. None of the early proponents of each theory survived to see the truth.

(Ref: LeCat: Concerning the foramen ovale being found open in the hearts of adults. Phil trans 9:134-135, 1747
Gintrac E: Observations and Researches on Cyanosis or Blue Disease. Paris: lmprime et Fonderie de J. Pinard, 1824)

In the 19th century, interest in the theoretical congenital cardiology evolved further with some of the great brains of the century rendering their interest in these. Important among them were:

Farre JR: On malformations of the human heart. In, Pathological Researches. London, 1814
Meckel JF: On malformations of the heart. Virchows Arch Pathol Anat 1805:594-610; 1815:221-284
Gintrac E: Observations and Researches on Cyanosis or Blue Disease. Paris: lmprime et Fonderie de J. Pinard, 1824
Paget: On the congenital malformations of the heart. Edinburgh Med Surg 36:263-309, 1831

These publications in the first half of the 19th century were only a prequel to a grand 2nd half. AD 1858 saw a monumental work by name “Malformations of the Human Heart” published in London, authored by one of the most celebrated physicians of the time, Sir Thomas Bevill Peacock. The book contained beautiful illustrations of various congenital malformations, including such things as ventricular septal defects, pulmonary stenosis, and transposition of the aorta and pulmonary artery, making it a pleasure to read. Even a person of Maude Abbott’s caliber exclaimed about Peacock’s book as, "the first comprehensive study covering the whole field (of Pediatric cardiology)". About 17 years later, another celebrated physician, Carl von Rokitansky published his monumental from Vienna, named “Defects of the Cardiac Septa”. Having personally done some 30,000 autopsies, Rokitansky was probably the best-suited person of the period to write anything related to Pathological Anatomy. No wonder, he was later called as “Linnaeus of
pathological anatomy” by Rudolf Virchow himself. Rokitansky was also a very keen observer of latest and could appreciate the best of the era. He, in fact, had openly supported Ignaz Philipp Semmelweis in his work on Puerperal fever. The works of Peacock and Rokitansky added to the knowledge of the existing cardiac disease to a great extent and inspired generations of physicians to look at the subject with keener eyes.

Peacock was famous not just for his publications, but also for his ability to accommodate to the new technology available in the period. He noted a characteristic radiation of the murmur of pulmonary stenosis using the new tool, the stethoscope, to correlate physical findings with anatomy in congenitally malformed hearts. He thus became a pioneer in the use of this device for heart, others of the era being more interested in lungs. Peacock was also interested in etiology. He was first to observe that "malformations are certainly most common in males, though why it should be so seems incapable of explanation." He also observed the familial nature of cardiac defects, presaged a long period of analysis of genetic and familial aspects of heart malformations, a process that continues using vastly more sophisticated tools to the present day.

(Ref: Peacock TB. On malformations of the human heart. London: John Churchill, 1858.

Peacock TB. On some of the causes and effects of valvular disease of the heart [together with] On the prognosis in cases of valvular disease of the heart. London: John Churchill, 1865. Reprinted in: Fye WB, ed. Birmingham, AL: The Classics of Cardiology Library, 1990

Rokitansky : Defects of the Cardiac Septa. Vienna, 1875)

Next time, we shall see the further development of 19th century.

On a personal note, the week saw an acutely short staffed and over-stressed Surgical staff. It is not easy for a person to do/assist 5 cases a day. I hope they will draw their strength from the work itself!

How partial can be an AV canal defect? We have seen an isolated primum defect. How about an intact interatrial septum with common AV valve with inlet VSD? We had a tough time differentiating this with a Tricuspid atresia, as the MRV was hypoplastic. Great vessels were normally related. The management may not change, but the diagnosis is obviously, a matter to dwell upon.

I was discussing the differentials of AortoPulmonary windows and explained the need for looking at both semilunar valves as a differentiating factor from Truncus. What I saw in a child was much intriguing. There were 2 semilunar valves with large communication between Aorta and MPA. The MPA continued towards LPA and stopped there. No distal LPA! RPA took separate origin from the Aorta, just above the Aorto-pulmonary communication. Technically, it was an Aortopulmonary window with Hemitruncus! I had not seen this combination and my previous publication of 50 cases of Aortopulmonay window also did not feature this entity. I think I should bring it to the notice of Dr Robert Anderson.

Please put in your inputs. If you happen to see anything that is similar what I discuss every time, feel free to comment or email me.

Regards

Kiran

1 comment:

  1. Nice to learn about two interesting cases.
    Keep it up sir.

    ReplyDelete